BACKGROUND Cardiac sarcoidosis(CS)is an infiltrative disease with manifestations such as nonsustained ventricular tachycardia(NSVT)and heart failure(HF).Antiphospholipid syndrome(APS)and antiphospholipid positivity(AP...BACKGROUND Cardiac sarcoidosis(CS)is an infiltrative disease with manifestations such as nonsustained ventricular tachycardia(NSVT)and heart failure(HF).Antiphospholipid syndrome(APS)and antiphospholipid positivity(APP)are prothrombotic phenomena which elevate risk for thromboembolism.CS with active systemic sarcoid and APS/APP is a rare combination of diseases.CASE SUMMARY A 54 year old male with HF presented with several cardiopulmonary symptoms.Chest imaging showed bilateral patchy and reticulonodular infiltrates.Subsequent lung biopsy confirmed pulmonary sarcoidosis.Positron emission tomography revealed active systemic sarcoidosis(SS)and fibrotic CS.Positive antiphospholipid antibodies without thromboembolism confirmed APP.HF and APP were managed with medical therapy.Fibrotic CS and NSVT required permanent cardiac device and antiarrhythmic therapy.SS was managed with early taper of steroids and transition to biologics.CONCLUSION Fibrotic CS with active SS and APS/APP has not been previously described in literature.This case utilized a modified approach for the management of this combination of diseases.As immunosuppressants such as steroids have limited utility in fibrotic sarcoidosis and a potential for thromboembolic complications in the presence of APP,an accelerated transition to non-thrombotic immunosuppressants can be advantageous in the long term treatment of this combination of diseases.展开更多
Cardiac sarcoidosis(CS)is a rare but serious manifestation of sarcoidosis that can lead to significant morbidity and mortality due to arrhythmias and heart failure.The inflammatory process in CS is characterized by th...Cardiac sarcoidosis(CS)is a rare but serious manifestation of sarcoidosis that can lead to significant morbidity and mortality due to arrhythmias and heart failure.The inflammatory process in CS is characterized by the formation of noncaseating granulomas in the myocardium,which can disrupt normal cardiac function and conduction.Corticosteroids are the primary therapeutic agents used to manage CS,particularly during the acute inflammatory phase,as they help reduce inflammation and improve cardiac function.However,the long-term use of steroids poses risks,including opportunistic infections and metabolic complications.Advanced imaging techniques,such as cardiac magnetic resonance imaging and positron emission tomography,play a crucial role in diagnosing CS and assessing myocardial involvement.These imaging modalities also aid in risk stratification for arrhythmic events,guiding therapeutic decisions such as the initiation of steroid therapy and the potential placement of implantable cardioverterdefibrillators.This review synthesizes current evidence regarding the role of steroid therapy in managing CS and its implications for cardiac arrhythmias,emphasizing the need for individualized treatment strategies to optimize patient outcomes.展开更多
BACKGROUND To evaluate the diagnostic utility of endobronchial ultrasound(EBUS)-guided mediastinal lymph node fenestration biopsy in atypical sarcoidosis using fine biopsy forceps[i.e.,EBUS-transbronchial forceps biop...BACKGROUND To evaluate the diagnostic utility of endobronchial ultrasound(EBUS)-guided mediastinal lymph node fenestration biopsy in atypical sarcoidosis using fine biopsy forceps[i.e.,EBUS-transbronchial forceps biopsy(TBFB)].CASE SUMMARY In this case series,two atypical sarcoidosis cases admitted in 2024 were retrospectively analyzed,both of whom lacked classical clinical manifestations.After chest computed tomography/positron emission tomography-computed tomography imaging and serum angiotensin-converting enzyme testing,EBUStransbronchial needle aspiration was performed using a 21-G needle.Subsequently,tissue sampling was performed at the enlarged puncture site by means of a 1.2-mm fine biopsy forceps.At the same time,bronchoalveolar lavage fluid lymphocyte subset analysis was conducted.CONCLUSION Both cases demonstrated non-caseating granulomatous inflammation on histopathology,elevated serum angiotensin-converting enzyme levels,and markedly increased CD4/CD8 ratios in bronchoalveolar lavage fluid.Case 1 was observed after confirmation of stage II sarcoidosis,whereas case 2 required glucocorticoid therapy due to the involvement of multiple systems.EBUS-TBFB can be used to make a definitive pathological diagnosis.For radiologically atypical sarcoidosis,EBUS-TBFB improves diagnostic accuracy by obtaining larger histological samples,thereby addressing the limitations of transbronchial needle aspiration cytology.This approach significantly improves differential diagnostic efficacy and holds substantial clinical relevance for broader adoption.展开更多
BACKGROUND Sarcoidosis is a rare multisystem disease characterized histologically by noncaseating granuloma formation in the affected organ.While cardiac sarcoidosis is found on autopsy in up to 25%of sarcoidosis case...BACKGROUND Sarcoidosis is a rare multisystem disease characterized histologically by noncaseating granuloma formation in the affected organ.While cardiac sarcoidosis is found on autopsy in up to 25%of sarcoidosis cases,it is still underdiagnosed and is associated with a poor prognosis.Although the etiology of sarcoidosis remains unclear,an antigen triggered exaggerated immune response has been hypothesized.Early detection and prompt management of cardiac sarcoidosis remains pivotal.CASE SUMMARY A 60-year-old female,with pulmonary sarcoidosis in remission,presented to the cardiology outpatient clinic for evaluation of weeks-long dyspnea on moderate exertion(New York Heart Association class II)that was relieved by rest.Submaximal exercise stress test showed multifocal ventricular extrasystoles,followed by a self-limiting torsades de pointes.Cardiac magnetic resonance imaging showed nondilated and normotrophic left ventricle with basoseptal and mid-septal dyskinesis.The magnetic resonance imaging-derived left ventricular ejection fraction was 45%.Delayed enhancement showed patchy transmural fibrosis of the septum and hyperenhancement of the papillary muscles,all in favor of extensive cardiac involvement of sarcoidosis.A double-chamber implantable cardiac defibrillator was implanted,and methylprednisolone(12 mg/d)and methotrexate(12.5 mg/wk)treatment was initiated.Follow-up and implantable cardiac defibrillator interrogation showed episodes of asymptomatic nonsustained ventricular tachycardia and an asymptomatic episode of nonsustained ventricular tachycardia ending by the first antitachycardia pacing run.CONCLUSION Along an extensive review of the literature,this unusual case report highlights the importance of early detection of cardiac involvement of sarcoidosis,in order to avoid potential complications and increase survival.展开更多
Sarcoidosis is a multisystem inflammatory granulomatous disease of unknown etiology. Neurosarcoidosis (NS) is a rare but potentially devastating manifestation of sarcoidosis, with a prevalence of approximately 5% in p...Sarcoidosis is a multisystem inflammatory granulomatous disease of unknown etiology. Neurosarcoidosis (NS) is a rare but potentially devastating manifestation of sarcoidosis, with a prevalence of approximately 5% in patients with systemic sarcoidosis. Due to the possible involvement of any part of the nervous system, a myriad of neurological manifestations can occur. Clinical features resulting from involvement of the hypothalmo-pituitary axis and cranial nerves, in particular, cranial nerve VII are the more common presentations of this condition. Medical therapy with corticosteroids is the mainstay of treatment and providing tissue for diagnosis remains the principal indication for surgery. Therapeutically, neurosurgery is indicated only for life-threatening complications. We describe the clinical case of a patient with fatally progressive NS who had multiple anesthetic exposures. This case highlights the perioperative considerations of NS and its anesthetic implications.展开更多
Purpose: To identify and interrogate the role of Gallium-67 scintigraphy in the diagnosis of Sarcoidosis. Methods: A retrospective study of 75 patients with suspected Sarcoidosis in a large teaching hospital with a sp...Purpose: To identify and interrogate the role of Gallium-67 scintigraphy in the diagnosis of Sarcoidosis. Methods: A retrospective study of 75 patients with suspected Sarcoidosis in a large teaching hospital with a specialist Sarcoidosis Centre. Results: Ga-67 scintigraphy in patients considered on clinical grounds to have Sarcoidosis is a useful and sensitive test, and furthermore, by relaxing the interpretation of gallium scintigraphy sensitivity may be increased (from 20% to 80%) for a relatively small drop in specificity. Conclusions: The sensitivity of diagnostic power may be increased with a change in diagnostic criteria at the expense of only a small drop in specificity. In the context of Neurosarcoidosis, our results support the theory that Ga-67 scintigraphy has value in identifying potential, asymptomatic biopsy sites outside of the CNS that may not be identified by other forms of imaging.展开更多
Sarcoidosis is a multisystem, chronic inflammatory disease that is characterized by the development of non-caseating granulomas in multiple body tissues and organ systems. Neurological complications of systemic sarcoi...Sarcoidosis is a multisystem, chronic inflammatory disease that is characterized by the development of non-caseating granulomas in multiple body tissues and organ systems. Neurological complications of systemic sarcoidosis include peripheral and cranial neuropathies, myopathies, seizures, gait dysfunction, and cognitive decline. Because sarcoidosis has a predilection to involve the basilar meninges, cranial neuropathy is the most prevalent neurological deficit seen when the nervous system is involved. Sarcoidosis cranial neuropathy may occur at different stages of the disease and even as the initial clinical manifestation of central nervous system involvement. Attributing a cranial neuropathy to sarcoidosis can be challenging, particularly in the setting of normal imaging studies. In this review, cranial neuropathies in sarcoidosis are discussed in detail.展开更多
A 39-year-old male reported fevers,weight loss,watery loose stools,and decreased visual acuity in his right eye over the prior five years.He was pancytopenic,had an elevated American council on exercise level,total bi...A 39-year-old male reported fevers,weight loss,watery loose stools,and decreased visual acuity in his right eye over the prior five years.He was pancytopenic,had an elevated American council on exercise level,total bilirubin,and alkaline phosphatase.Computed tomography revealed massive hepatosplenomegaly and emphysematous lung changes.Liver biopsy showed non caseating granulomas.The patient was diagnosed with extrapulmonary sarcoidosis and was treated with prednisone.The patient symptomatically improved but 5 mo later presented with abdominal pain caused by perforation of the cecum.He underwent a cecectomy and pathology revealed pneumatosis cystoides intestinalis.This represents the first reported association between pneumatosis cystoides intestinalis and sarcoidosis.The etiology of pneumatosis cystoides intestinalis in this case was likely multifactorial and involved both effects of the corticosteroids as well as the advanced nature of the gastrointestinal sarcoidosis.Furthermore this case has the unique features of emphysematous lung changes and pancytopenia which are uncommon with sarcoidosis.展开更多
Gastric sarcoidosis with noncaseating granuloma is rare. Although corticosteroid produces a dramatic clinical response, it is unknown whether azathioprine show efficacy in prednisolone-dependent cases. Here, we report...Gastric sarcoidosis with noncaseating granuloma is rare. Although corticosteroid produces a dramatic clinical response, it is unknown whether azathioprine show efficacy in prednisolone-dependent cases. Here, we report a case of gastric sarcoidosis in a 25-year-old man with severe epigastlargia. Gastroendoscopy revealed multiple map-like ulcerations. Histological examination showed multiple noncaseating granulomatous lesions in gastric mucosa, which were incompatible with diagnoses of Crohn's disease or tuberculosis. He was started on prednisolone at 30 mg/d, and his symptoms improved within 7-d. The prednisolone was gradually tapered by 5 mg every 2-wk, but oral azathioprine at 50 mg was added after symptoms recurred at tapered dose of 10 mg. Endoscopy 4-wk later showed healing ulcers, and, lymphocytic infiltration was absent. The efficacy of additional azathioprine in gastric sarcoidosis is not well defined. Here, we report a case of prednisolone-dependent gastric sarcoidosis that improved after additional azathioprine, and also review the literature concerning the treatment, especially for prednisolone-dependent cases.展开更多
BACKGROUND Sarcoidosis is a granulomatous disease of unknown etiology that most often impacts the lungs.Cutaneous manifestations of sarcoidosis are seen among 9%- 37% of patients.Subcutaneous sarcoidosis is a rare pre...BACKGROUND Sarcoidosis is a granulomatous disease of unknown etiology that most often impacts the lungs.Cutaneous manifestations of sarcoidosis are seen among 9%- 37% of patients.Subcutaneous sarcoidosis is a rare presentation of cutaneous sarcoidosis with estimates of frequency ranging from 1.4%-16%.To date,very few articles and case reports have been written about this subject.In this paper,we describe a case of subcutaneous sarcoidosis and perform a review of the literature to determine if there are commonalities among patients who present with subcutaneous sarcoidosis.CASE SUMMARY A 38-year-old female,with a past medical history of arthritis and recurrent nephrolithiasis,presents with an 8-mo history of 4 firm,asymptomatic,skincolored nodules on her left and right upper extremities and neck.Needle biopsy and post-excisional pathology report both revealed well-formed,dense,noncaseating granulomas localized to the subcutaneous tissue.Chest computed tomography revealed mild mediastinal lymphadenopathy.A diagnosis of subcutaneous sarcoidosis was made,and the lesions were surgically removed.CONCLUSION Commonalities among patients presenting with subcutaneous sarcoidosis include: middle-aged female,lesions localizing to the upper or lower limbs,lymphadenopathy or pulmonary infiltration on chest imaging,elevated serum angiotensin-converting enzyme.展开更多
Background： The purpose of this study was to describe our experiences and analyze the benefits of video-assisted thoracoscopic surgery （VATS） combined with positron emitted tomography （PET）-computed tomography ...Background： The purpose of this study was to describe our experiences and analyze the benefits of video-assisted thoracoscopic surgery （VATS） combined with positron emitted tomography （PET）-computed tomography （CT） in the diagnosis of patients with early （Stage 1） sarcoidosis. Methods： From 1995 to 2006, seven patients （two males, five females）, with ages ranging from 26 to 58 years, were impressed with Stage 1 sarcoidosis （mediastinal or hilar lymph nodes involvements without lung involvement） by histological examination of intrathoracic lymph nodes （LNs） and/or lung parenchyma taken＇from VATS biopsy. Three of them received PET or PET-CT evaluation. VATS was approached from the right and left side in one and six patients, respectively, according to the locations of their lesions. Results： All the VATS biopsied LNs or lung specimens were adequate for establishing diagnosis. Mediastinal LNs were taken from Groups 3, 4 in four, Group 7 in two, and Groups 5, 6 in one of them. Hilar LNs biopsies were performed in four cases. Lung biopsy was performed in all but two cases. All of them were expressed pathologically or radiologically as Stage 1 sarcoidosis. PET-CT revealed high emission signals over these affected LNs. These patients received oral steroid treatment or follow up only. All of them were followed up from 5 months to 11 years with satisfactory results. Conclusion： VATS biopsy is a minimally invasive, safe and effective procedure. It can be used as a diagnostic altermative of transbronchial lung biopsy （TBLB）, and can harvest larger and more areas of specimens than mediastinoscopy for staging patients with sarcoidosis. PET-CT can provide us more accurate information about the characteristics and localization of these lesions before biopsy. VATS combined with PET-CT can provide more accurate and earlier diagnosis of patients with unknown intrathoracic lesions, including the sarcoidosis.展开更多
Sarcoidosis is a systemic disease of unknown aetiology that may affect any organ in the body. The gastrointestinal tract however is only rarely affected outside the liver. Symptoms may be non-specific. Irritable b...Sarcoidosis is a systemic disease of unknown aetiology that may affect any organ in the body. The gastrointestinal tract however is only rarely affected outside the liver. Symptoms may be non-specific. Irritable bowel syndrome （IBS） is a common diagnosis. The recognition of IBS is aided by the use of the Rome Ⅱ criteria-in the absence of organic disease. We describe the first case of a patient with gastric sarcoidosis who presented with IBS symptoms but subsequently responded to immunosuppressive therapy.展开更多
Cardiac involvement of sarcoid lesions is diagnosed by myocardial biopsy which is frequently false-negative,and patients with cardiac sarcoidosis(CS) who have impaired left ventricular(LV) systolic function are someti...Cardiac involvement of sarcoid lesions is diagnosed by myocardial biopsy which is frequently false-negative,and patients with cardiac sarcoidosis(CS) who have impaired left ventricular(LV) systolic function are sometimes diagnosed with dilated cardiomyopathy(DCM).Late gadolinium enhancement(LE) in magnetic resonance imaging is now a critical finding in diagnosing CS,and the novel Japanese guideline considers myocardial LE to be a major criterion of CS.This article describes the value of LE in patients with CS who have impaired LV systolic function,particularly the diagnostic and clinical significance of LE distribution in comparison with DCM.LE existed at all LV segments and myocardial layers in patients with CS,whereas it was localized predominantly in the midwall of basal to mid septum in those with DCM.Transmural(nodular),circumferential,and subepicardial and subendocardial LE distribution were highly specific in patients with CS,whereas the prevalence of striated midwall LE were high both in patients with CS and with DCM.Since sarcoidosis patients with LE have higher incidences of heart failure symptoms,ventricular tachyarrhythmia and sudden cardiac death,the analyses of extent and distribution of LE are crucial in early diagnosis and therapeutic approach for patients with CS.展开更多
Since it has been recognized that sarcoidosis(SA) is not an exclusive disorder of the lungs but can also affect other organs such as the liver and spleen, efforts have been made to define specific imaging criteria for...Since it has been recognized that sarcoidosis(SA) is not an exclusive disorder of the lungs but can also affect other organs such as the liver and spleen, efforts have been made to define specific imaging criteria for the diagnosis of the single organ involvement, and the concept has been reinforced that the exclusion of alternative causes is important to achieve the correct diagnosis. Ultrasound(US)is a useful tool to evaluate patients with suspected abdominal SA, such as of the liver, spleen, kidney, pancreas and other organs, showing findings such as organomegaly, focal lesions and lymphadenopathy. While the diagnosis of abdominal SA is more predictable in the case of involvement of other organs(e.g.,lungs), the problem is more complex in the case of isolated abdominal SA. The recent use of contrast-enhanced ultrasound and endoscopic ultrasound elastography has provided additional information about the enhancement patterns and tissue rigidity in abdominal SA. Here we critically review the role of US in abdominal SA, reporting typical findings and limitations of current evidence and by discussing future perspectives of study.展开更多
Sarcoidosis is a benign systematic granulomatous disorder of unknown etiology and is associated with various malignancies.However, granulomatous and metastatic lymph node lesions are difficult to distinguish even when...Sarcoidosis is a benign systematic granulomatous disorder of unknown etiology and is associated with various malignancies.However, granulomatous and metastatic lymph node lesions are difficult to distinguish even when using precise and modern diagnostic methods, such as positron emission tomography. Thus, histological verification is the only method that can be used to accurately describe the nature of this disease. In this article, we report a case of non-luminal HER-2/neu-positive breast cancer in a patient without history of sarcoidosis and suspected to have metastatic disease.展开更多
Sarcoidosis is a systemic granulomatous disease which may involve many organs.In approximately 95% of patients there is liver involvement,with noncaseating hepatic granulomas occurring in 21 to 99% of patients with sa...Sarcoidosis is a systemic granulomatous disease which may involve many organs.In approximately 95% of patients there is liver involvement,with noncaseating hepatic granulomas occurring in 21 to 99% of patients with sarcoidosis.Liver involvement is usually asymptomatic and limited to mild to moderate abnormalities in liver biochemistry.The occurrence of jaundice in sarcoidosis is rare;extensive imaging procedures and the examination of liver biopsies permit a precise diagnostic.Ductopenia associated with sarcoidosis has been reported in less than 20 cases and can lead to biliary cirrhosis and liver-related death.We report here on a case of ductopenia-related sarcoidosis in which primary biliary cirrhosis and extrahepatic cholestasis have been carefully excluded.The patient follow up was 8 years.Although ursodesoxycholic acid appears to improve liver biochemistry it does not preclude the rapid occurrence of extensive fibrosis.A review of the literature of reported cases of ductopenia related to sarcoidosis is provided.展开更多
A 39-year-old male patient complaining of bilateral hand joint arthralgia was evaluated and found to have chronic hepatitis C and systemic sarcoidosis involving lung, skin, liver, and spleen. Hepatic and cutaneous sar...A 39-year-old male patient complaining of bilateral hand joint arthralgia was evaluated and found to have chronic hepatitis C and systemic sarcoidosis involving lung, skin, liver, and spleen. Hepatic and cutaneous sarcoidoses were confirmed by the presence of numerous noncaseating granulomas on histological examination. Pulmonary and splenic involvements were diagnosed by imaging studies. Fifteen months later, the sarcoidotic lesions in lung, liver, and spleen were resolved by radiological studies and a liver biopsy showed no granuloma but moderate to severe inflammatory activity, systemic sarcoidosis is a rare comorbidity of chronic hepatitis C which may spontaneously resolve.展开更多
BACKGROUND Rearrangements of the anaplastic lymphoma kinase(ALK)gene(ALK-positive)represent an oncogenic driver in approximately 3%-5%of non-small-lung cancer(NSCLC)patients.Sarcoidosis is a multisystem disease,and it...BACKGROUND Rearrangements of the anaplastic lymphoma kinase(ALK)gene(ALK-positive)represent an oncogenic driver in approximately 3%-5%of non-small-lung cancer(NSCLC)patients.Sarcoidosis is a multisystem disease,and its reported incidence in Asia is 1 or less per 100000 people per year.The co-occurrence of sarcoidosis and ALK-positive NSCLC is rare,and ALK-positive lung cancer is likely to spread quickly.Therefore,the co-occurrence of sarcoidosis is more easily misdiagnosed as metastatic lung cancer by radiological examination.CASE SUMMARY A 50-year-old man had a nodule in the left superior lobe,many small nodules in left superior and right lungs,and enlarged bilateral hilar,mediastinal,and right supraclavicular lymph nodes.Computed tomography-guided pulmonary biopsy of the nodule in the left superior lobe revealed echinoderm microtubuleassociated protein-like 4 gene-ALK positive NSCLC with concomitant noncaseating granuloma.This patient was treated with crizotinib.Thirty days later,a chest computed tomography scan revealed a dramatic decrease in the size of the left superior lobe nodule;however,the lesions in the right lung progressed.The right supraclavicular lymph nodes showed granulomas,and no tumor cells were identified in the specimens. The angiotensin-converting enzyme level was high.After 1 wk of methylprednisolone treatment, a significant response of all lesionswas revealed. Following radical resection of the lung cancer, noncaseatinggranulomas were observed in both lung tissues and lymph nodes, which resultedin a diagnosis of echinoderm microtubule-associated protein-like 4-ALK positiveNSCLC accompanied with sarcoidosis.CONCLUSIONOur experience illustrates that pathological evidence is needed to confirmmetastatic disease, especially when some suspected metastatic lesions arenegative for malignancy.展开更多
Polymerase chain reaction (PCR) was used to detect the presence of Barrelia burgdoferi DNA in biological samples from patients with sarcoidosis. The target DNA sequence was of chromosomal origin. The amplified DNA seq...Polymerase chain reaction (PCR) was used to detect the presence of Barrelia burgdoferi DNA in biological samples from patients with sarcoidosis. The target DNA sequence was of chromosomal origin. The amplified DNA sequence was analyzed by agarose gel electrophoresis, PAGE with silver staining, and the identity of amplified DNA was confirmed by restriction enzyme cleavage and DNA-DNA hybridization with a 32P-labelled probe. The assay was sensitive to fewer than two copies of B. burgdorferi genome, even in the presence of a 104-fold excess of human eukaryotic DNA , and was also specific to different B. burgdorferi strains tested. Sera serologically positive to B. burgdcrferi (n=26), bronchoalveolar lavage fluid and supernatant of BALF (n=26) and peripheral blood (n=9) from sarcoidosis patients were tested. The positive rate was low (4/26, 2/26. and 0/9, respectively). It was considered that DNA from B. burgdor ferimay be identified in a minority of patients with sarcoidosis, and it may play a pathogenetic role in such cases. More studies need to be done before advancing the hypothesis of an etiologic role of B. burgdorferi in sarcoidosis.展开更多
Dear Editor,We report a case of Purtscher-like retinopathy(PLR)in the presence of acute pancreatitis secondary to sarcoidosis.To our knowledge,this is the first case report of a PLR in a patient with sarcoidosis.
文摘BACKGROUND Cardiac sarcoidosis(CS)is an infiltrative disease with manifestations such as nonsustained ventricular tachycardia(NSVT)and heart failure(HF).Antiphospholipid syndrome(APS)and antiphospholipid positivity(APP)are prothrombotic phenomena which elevate risk for thromboembolism.CS with active systemic sarcoid and APS/APP is a rare combination of diseases.CASE SUMMARY A 54 year old male with HF presented with several cardiopulmonary symptoms.Chest imaging showed bilateral patchy and reticulonodular infiltrates.Subsequent lung biopsy confirmed pulmonary sarcoidosis.Positron emission tomography revealed active systemic sarcoidosis(SS)and fibrotic CS.Positive antiphospholipid antibodies without thromboembolism confirmed APP.HF and APP were managed with medical therapy.Fibrotic CS and NSVT required permanent cardiac device and antiarrhythmic therapy.SS was managed with early taper of steroids and transition to biologics.CONCLUSION Fibrotic CS with active SS and APS/APP has not been previously described in literature.This case utilized a modified approach for the management of this combination of diseases.As immunosuppressants such as steroids have limited utility in fibrotic sarcoidosis and a potential for thromboembolic complications in the presence of APP,an accelerated transition to non-thrombotic immunosuppressants can be advantageous in the long term treatment of this combination of diseases.
文摘Cardiac sarcoidosis(CS)is a rare but serious manifestation of sarcoidosis that can lead to significant morbidity and mortality due to arrhythmias and heart failure.The inflammatory process in CS is characterized by the formation of noncaseating granulomas in the myocardium,which can disrupt normal cardiac function and conduction.Corticosteroids are the primary therapeutic agents used to manage CS,particularly during the acute inflammatory phase,as they help reduce inflammation and improve cardiac function.However,the long-term use of steroids poses risks,including opportunistic infections and metabolic complications.Advanced imaging techniques,such as cardiac magnetic resonance imaging and positron emission tomography,play a crucial role in diagnosing CS and assessing myocardial involvement.These imaging modalities also aid in risk stratification for arrhythmic events,guiding therapeutic decisions such as the initiation of steroid therapy and the potential placement of implantable cardioverterdefibrillators.This review synthesizes current evidence regarding the role of steroid therapy in managing CS and its implications for cardiac arrhythmias,emphasizing the need for individualized treatment strategies to optimize patient outcomes.
基金Supported by the National Natural Science Foundation of China,No.82170103Natural Science Foundation of Fujian Province,No.2024J011325+1 种基金Young People Training Project from Fujian Province Health Bureau,No.2020GGB057 and No.2023QNB008Xiamen Medical and Health Guidance Project,No.3502Z20224ZD1060,No.3502Z20214ZD1043,and No.3502Z20224ZD1058.
文摘BACKGROUND To evaluate the diagnostic utility of endobronchial ultrasound(EBUS)-guided mediastinal lymph node fenestration biopsy in atypical sarcoidosis using fine biopsy forceps[i.e.,EBUS-transbronchial forceps biopsy(TBFB)].CASE SUMMARY In this case series,two atypical sarcoidosis cases admitted in 2024 were retrospectively analyzed,both of whom lacked classical clinical manifestations.After chest computed tomography/positron emission tomography-computed tomography imaging and serum angiotensin-converting enzyme testing,EBUStransbronchial needle aspiration was performed using a 21-G needle.Subsequently,tissue sampling was performed at the enlarged puncture site by means of a 1.2-mm fine biopsy forceps.At the same time,bronchoalveolar lavage fluid lymphocyte subset analysis was conducted.CONCLUSION Both cases demonstrated non-caseating granulomatous inflammation on histopathology,elevated serum angiotensin-converting enzyme levels,and markedly increased CD4/CD8 ratios in bronchoalveolar lavage fluid.Case 1 was observed after confirmation of stage II sarcoidosis,whereas case 2 required glucocorticoid therapy due to the involvement of multiple systems.EBUS-TBFB can be used to make a definitive pathological diagnosis.For radiologically atypical sarcoidosis,EBUS-TBFB improves diagnostic accuracy by obtaining larger histological samples,thereby addressing the limitations of transbronchial needle aspiration cytology.This approach significantly improves differential diagnostic efficacy and holds substantial clinical relevance for broader adoption.
文摘BACKGROUND Sarcoidosis is a rare multisystem disease characterized histologically by noncaseating granuloma formation in the affected organ.While cardiac sarcoidosis is found on autopsy in up to 25%of sarcoidosis cases,it is still underdiagnosed and is associated with a poor prognosis.Although the etiology of sarcoidosis remains unclear,an antigen triggered exaggerated immune response has been hypothesized.Early detection and prompt management of cardiac sarcoidosis remains pivotal.CASE SUMMARY A 60-year-old female,with pulmonary sarcoidosis in remission,presented to the cardiology outpatient clinic for evaluation of weeks-long dyspnea on moderate exertion(New York Heart Association class II)that was relieved by rest.Submaximal exercise stress test showed multifocal ventricular extrasystoles,followed by a self-limiting torsades de pointes.Cardiac magnetic resonance imaging showed nondilated and normotrophic left ventricle with basoseptal and mid-septal dyskinesis.The magnetic resonance imaging-derived left ventricular ejection fraction was 45%.Delayed enhancement showed patchy transmural fibrosis of the septum and hyperenhancement of the papillary muscles,all in favor of extensive cardiac involvement of sarcoidosis.A double-chamber implantable cardiac defibrillator was implanted,and methylprednisolone(12 mg/d)and methotrexate(12.5 mg/wk)treatment was initiated.Follow-up and implantable cardiac defibrillator interrogation showed episodes of asymptomatic nonsustained ventricular tachycardia and an asymptomatic episode of nonsustained ventricular tachycardia ending by the first antitachycardia pacing run.CONCLUSION Along an extensive review of the literature,this unusual case report highlights the importance of early detection of cardiac involvement of sarcoidosis,in order to avoid potential complications and increase survival.
文摘Sarcoidosis is a multisystem inflammatory granulomatous disease of unknown etiology. Neurosarcoidosis (NS) is a rare but potentially devastating manifestation of sarcoidosis, with a prevalence of approximately 5% in patients with systemic sarcoidosis. Due to the possible involvement of any part of the nervous system, a myriad of neurological manifestations can occur. Clinical features resulting from involvement of the hypothalmo-pituitary axis and cranial nerves, in particular, cranial nerve VII are the more common presentations of this condition. Medical therapy with corticosteroids is the mainstay of treatment and providing tissue for diagnosis remains the principal indication for surgery. Therapeutically, neurosurgery is indicated only for life-threatening complications. We describe the clinical case of a patient with fatally progressive NS who had multiple anesthetic exposures. This case highlights the perioperative considerations of NS and its anesthetic implications.
文摘Purpose: To identify and interrogate the role of Gallium-67 scintigraphy in the diagnosis of Sarcoidosis. Methods: A retrospective study of 75 patients with suspected Sarcoidosis in a large teaching hospital with a specialist Sarcoidosis Centre. Results: Ga-67 scintigraphy in patients considered on clinical grounds to have Sarcoidosis is a useful and sensitive test, and furthermore, by relaxing the interpretation of gallium scintigraphy sensitivity may be increased (from 20% to 80%) for a relatively small drop in specificity. Conclusions: The sensitivity of diagnostic power may be increased with a change in diagnostic criteria at the expense of only a small drop in specificity. In the context of Neurosarcoidosis, our results support the theory that Ga-67 scintigraphy has value in identifying potential, asymptomatic biopsy sites outside of the CNS that may not be identified by other forms of imaging.
文摘Sarcoidosis is a multisystem, chronic inflammatory disease that is characterized by the development of non-caseating granulomas in multiple body tissues and organ systems. Neurological complications of systemic sarcoidosis include peripheral and cranial neuropathies, myopathies, seizures, gait dysfunction, and cognitive decline. Because sarcoidosis has a predilection to involve the basilar meninges, cranial neuropathy is the most prevalent neurological deficit seen when the nervous system is involved. Sarcoidosis cranial neuropathy may occur at different stages of the disease and even as the initial clinical manifestation of central nervous system involvement. Attributing a cranial neuropathy to sarcoidosis can be challenging, particularly in the setting of normal imaging studies. In this review, cranial neuropathies in sarcoidosis are discussed in detail.
文摘A 39-year-old male reported fevers,weight loss,watery loose stools,and decreased visual acuity in his right eye over the prior five years.He was pancytopenic,had an elevated American council on exercise level,total bilirubin,and alkaline phosphatase.Computed tomography revealed massive hepatosplenomegaly and emphysematous lung changes.Liver biopsy showed non caseating granulomas.The patient was diagnosed with extrapulmonary sarcoidosis and was treated with prednisone.The patient symptomatically improved but 5 mo later presented with abdominal pain caused by perforation of the cecum.He underwent a cecectomy and pathology revealed pneumatosis cystoides intestinalis.This represents the first reported association between pneumatosis cystoides intestinalis and sarcoidosis.The etiology of pneumatosis cystoides intestinalis in this case was likely multifactorial and involved both effects of the corticosteroids as well as the advanced nature of the gastrointestinal sarcoidosis.Furthermore this case has the unique features of emphysematous lung changes and pancytopenia which are uncommon with sarcoidosis.
文摘Gastric sarcoidosis with noncaseating granuloma is rare. Although corticosteroid produces a dramatic clinical response, it is unknown whether azathioprine show efficacy in prednisolone-dependent cases. Here, we report a case of gastric sarcoidosis in a 25-year-old man with severe epigastlargia. Gastroendoscopy revealed multiple map-like ulcerations. Histological examination showed multiple noncaseating granulomatous lesions in gastric mucosa, which were incompatible with diagnoses of Crohn's disease or tuberculosis. He was started on prednisolone at 30 mg/d, and his symptoms improved within 7-d. The prednisolone was gradually tapered by 5 mg every 2-wk, but oral azathioprine at 50 mg was added after symptoms recurred at tapered dose of 10 mg. Endoscopy 4-wk later showed healing ulcers, and, lymphocytic infiltration was absent. The efficacy of additional azathioprine in gastric sarcoidosis is not well defined. Here, we report a case of prednisolone-dependent gastric sarcoidosis that improved after additional azathioprine, and also review the literature concerning the treatment, especially for prednisolone-dependent cases.
文摘BACKGROUND Sarcoidosis is a granulomatous disease of unknown etiology that most often impacts the lungs.Cutaneous manifestations of sarcoidosis are seen among 9%- 37% of patients.Subcutaneous sarcoidosis is a rare presentation of cutaneous sarcoidosis with estimates of frequency ranging from 1.4%-16%.To date,very few articles and case reports have been written about this subject.In this paper,we describe a case of subcutaneous sarcoidosis and perform a review of the literature to determine if there are commonalities among patients who present with subcutaneous sarcoidosis.CASE SUMMARY A 38-year-old female,with a past medical history of arthritis and recurrent nephrolithiasis,presents with an 8-mo history of 4 firm,asymptomatic,skincolored nodules on her left and right upper extremities and neck.Needle biopsy and post-excisional pathology report both revealed well-formed,dense,noncaseating granulomas localized to the subcutaneous tissue.Chest computed tomography revealed mild mediastinal lymphadenopathy.A diagnosis of subcutaneous sarcoidosis was made,and the lesions were surgically removed.CONCLUSION Commonalities among patients presenting with subcutaneous sarcoidosis include: middle-aged female,lesions localizing to the upper or lower limbs,lymphadenopathy or pulmonary infiltration on chest imaging,elevated serum angiotensin-converting enzyme.
文摘Background： The purpose of this study was to describe our experiences and analyze the benefits of video-assisted thoracoscopic surgery （VATS） combined with positron emitted tomography （PET）-computed tomography （CT） in the diagnosis of patients with early （Stage 1） sarcoidosis. Methods： From 1995 to 2006, seven patients （two males, five females）, with ages ranging from 26 to 58 years, were impressed with Stage 1 sarcoidosis （mediastinal or hilar lymph nodes involvements without lung involvement） by histological examination of intrathoracic lymph nodes （LNs） and/or lung parenchyma taken＇from VATS biopsy. Three of them received PET or PET-CT evaluation. VATS was approached from the right and left side in one and six patients, respectively, according to the locations of their lesions. Results： All the VATS biopsied LNs or lung specimens were adequate for establishing diagnosis. Mediastinal LNs were taken from Groups 3, 4 in four, Group 7 in two, and Groups 5, 6 in one of them. Hilar LNs biopsies were performed in four cases. Lung biopsy was performed in all but two cases. All of them were expressed pathologically or radiologically as Stage 1 sarcoidosis. PET-CT revealed high emission signals over these affected LNs. These patients received oral steroid treatment or follow up only. All of them were followed up from 5 months to 11 years with satisfactory results. Conclusion： VATS biopsy is a minimally invasive, safe and effective procedure. It can be used as a diagnostic altermative of transbronchial lung biopsy （TBLB）, and can harvest larger and more areas of specimens than mediastinoscopy for staging patients with sarcoidosis. PET-CT can provide us more accurate information about the characteristics and localization of these lesions before biopsy. VATS combined with PET-CT can provide more accurate and earlier diagnosis of patients with unknown intrathoracic lesions, including the sarcoidosis.
文摘Sarcoidosis is a systemic disease of unknown aetiology that may affect any organ in the body. The gastrointestinal tract however is only rarely affected outside the liver. Symptoms may be non-specific. Irritable bowel syndrome （IBS） is a common diagnosis. The recognition of IBS is aided by the use of the Rome Ⅱ criteria-in the absence of organic disease. We describe the first case of a patient with gastric sarcoidosis who presented with IBS symptoms but subsequently responded to immunosuppressive therapy.
文摘Cardiac involvement of sarcoid lesions is diagnosed by myocardial biopsy which is frequently false-negative,and patients with cardiac sarcoidosis(CS) who have impaired left ventricular(LV) systolic function are sometimes diagnosed with dilated cardiomyopathy(DCM).Late gadolinium enhancement(LE) in magnetic resonance imaging is now a critical finding in diagnosing CS,and the novel Japanese guideline considers myocardial LE to be a major criterion of CS.This article describes the value of LE in patients with CS who have impaired LV systolic function,particularly the diagnostic and clinical significance of LE distribution in comparison with DCM.LE existed at all LV segments and myocardial layers in patients with CS,whereas it was localized predominantly in the midwall of basal to mid septum in those with DCM.Transmural(nodular),circumferential,and subepicardial and subendocardial LE distribution were highly specific in patients with CS,whereas the prevalence of striated midwall LE were high both in patients with CS and with DCM.Since sarcoidosis patients with LE have higher incidences of heart failure symptoms,ventricular tachyarrhythmia and sudden cardiac death,the analyses of extent and distribution of LE are crucial in early diagnosis and therapeutic approach for patients with CS.
文摘Since it has been recognized that sarcoidosis(SA) is not an exclusive disorder of the lungs but can also affect other organs such as the liver and spleen, efforts have been made to define specific imaging criteria for the diagnosis of the single organ involvement, and the concept has been reinforced that the exclusion of alternative causes is important to achieve the correct diagnosis. Ultrasound(US)is a useful tool to evaluate patients with suspected abdominal SA, such as of the liver, spleen, kidney, pancreas and other organs, showing findings such as organomegaly, focal lesions and lymphadenopathy. While the diagnosis of abdominal SA is more predictable in the case of involvement of other organs(e.g.,lungs), the problem is more complex in the case of isolated abdominal SA. The recent use of contrast-enhanced ultrasound and endoscopic ultrasound elastography has provided additional information about the enhancement patterns and tissue rigidity in abdominal SA. Here we critically review the role of US in abdominal SA, reporting typical findings and limitations of current evidence and by discussing future perspectives of study.
文摘Sarcoidosis is a benign systematic granulomatous disorder of unknown etiology and is associated with various malignancies.However, granulomatous and metastatic lymph node lesions are difficult to distinguish even when using precise and modern diagnostic methods, such as positron emission tomography. Thus, histological verification is the only method that can be used to accurately describe the nature of this disease. In this article, we report a case of non-luminal HER-2/neu-positive breast cancer in a patient without history of sarcoidosis and suspected to have metastatic disease.
文摘Sarcoidosis is a systemic granulomatous disease which may involve many organs.In approximately 95% of patients there is liver involvement,with noncaseating hepatic granulomas occurring in 21 to 99% of patients with sarcoidosis.Liver involvement is usually asymptomatic and limited to mild to moderate abnormalities in liver biochemistry.The occurrence of jaundice in sarcoidosis is rare;extensive imaging procedures and the examination of liver biopsies permit a precise diagnostic.Ductopenia associated with sarcoidosis has been reported in less than 20 cases and can lead to biliary cirrhosis and liver-related death.We report here on a case of ductopenia-related sarcoidosis in which primary biliary cirrhosis and extrahepatic cholestasis have been carefully excluded.The patient follow up was 8 years.Although ursodesoxycholic acid appears to improve liver biochemistry it does not preclude the rapid occurrence of extensive fibrosis.A review of the literature of reported cases of ductopenia related to sarcoidosis is provided.
文摘A 39-year-old male patient complaining of bilateral hand joint arthralgia was evaluated and found to have chronic hepatitis C and systemic sarcoidosis involving lung, skin, liver, and spleen. Hepatic and cutaneous sarcoidoses were confirmed by the presence of numerous noncaseating granulomas on histological examination. Pulmonary and splenic involvements were diagnosed by imaging studies. Fifteen months later, the sarcoidotic lesions in lung, liver, and spleen were resolved by radiological studies and a liver biopsy showed no granuloma but moderate to severe inflammatory activity, systemic sarcoidosis is a rare comorbidity of chronic hepatitis C which may spontaneously resolve.
基金The Zhejiang Provincial Natural Science Foundation of China,No.LQ17H160005.
文摘BACKGROUND Rearrangements of the anaplastic lymphoma kinase(ALK)gene(ALK-positive)represent an oncogenic driver in approximately 3%-5%of non-small-lung cancer(NSCLC)patients.Sarcoidosis is a multisystem disease,and its reported incidence in Asia is 1 or less per 100000 people per year.The co-occurrence of sarcoidosis and ALK-positive NSCLC is rare,and ALK-positive lung cancer is likely to spread quickly.Therefore,the co-occurrence of sarcoidosis is more easily misdiagnosed as metastatic lung cancer by radiological examination.CASE SUMMARY A 50-year-old man had a nodule in the left superior lobe,many small nodules in left superior and right lungs,and enlarged bilateral hilar,mediastinal,and right supraclavicular lymph nodes.Computed tomography-guided pulmonary biopsy of the nodule in the left superior lobe revealed echinoderm microtubuleassociated protein-like 4 gene-ALK positive NSCLC with concomitant noncaseating granuloma.This patient was treated with crizotinib.Thirty days later,a chest computed tomography scan revealed a dramatic decrease in the size of the left superior lobe nodule;however,the lesions in the right lung progressed.The right supraclavicular lymph nodes showed granulomas,and no tumor cells were identified in the specimens. The angiotensin-converting enzyme level was high.After 1 wk of methylprednisolone treatment, a significant response of all lesionswas revealed. Following radical resection of the lung cancer, noncaseatinggranulomas were observed in both lung tissues and lymph nodes, which resultedin a diagnosis of echinoderm microtubule-associated protein-like 4-ALK positiveNSCLC accompanied with sarcoidosis.CONCLUSIONOur experience illustrates that pathological evidence is needed to confirmmetastatic disease, especially when some suspected metastatic lesions arenegative for malignancy.
文摘Polymerase chain reaction (PCR) was used to detect the presence of Barrelia burgdoferi DNA in biological samples from patients with sarcoidosis. The target DNA sequence was of chromosomal origin. The amplified DNA sequence was analyzed by agarose gel electrophoresis, PAGE with silver staining, and the identity of amplified DNA was confirmed by restriction enzyme cleavage and DNA-DNA hybridization with a 32P-labelled probe. The assay was sensitive to fewer than two copies of B. burgdorferi genome, even in the presence of a 104-fold excess of human eukaryotic DNA , and was also specific to different B. burgdorferi strains tested. Sera serologically positive to B. burgdcrferi (n=26), bronchoalveolar lavage fluid and supernatant of BALF (n=26) and peripheral blood (n=9) from sarcoidosis patients were tested. The positive rate was low (4/26, 2/26. and 0/9, respectively). It was considered that DNA from B. burgdor ferimay be identified in a minority of patients with sarcoidosis, and it may play a pathogenetic role in such cases. More studies need to be done before advancing the hypothesis of an etiologic role of B. burgdorferi in sarcoidosis.
文摘Dear Editor,We report a case of Purtscher-like retinopathy(PLR)in the presence of acute pancreatitis secondary to sarcoidosis.To our knowledge,this is the first case report of a PLR in a patient with sarcoidosis.
