BACKGROUND Immunoglobulin G4-related disease(IgG4-RD)is a multi-system fibroinflammatory disorder that can involve any organ,including the salivary glands,pancreas,and biliary tree.Treatment of immunoglobulin G4-relat...BACKGROUND Immunoglobulin G4-related disease(IgG4-RD)is a multi-system fibroinflammatory disorder that can involve any organ,including the salivary glands,pancreas,and biliary tree.Treatment of immunoglobulin G4-related sclerosing cholangitis(IgG4-SC)is similar to that for IgG4-RD,but progression is irreversible in some cases.We present a case of IgG4-SC in which an immuno-suppressant induced marked clinical and radiologic improvement.CASE SUMMARY A 63-year-old male presented with a prominent itching sensation and wholebody jaundice.He showed obstructive-pattern jaundice,an elevated IgG4 level,and infiltration of a large number of IgG4-positive cells in the ampulla of Vater.The imaging findings of intrahepatic duct(IHD)and common bile duct dilation,an elevated serum IgG4 level,and characteristic histological findings led to diagnosis of IgG4-SC that compatible with the 2019 ACR/EULAR classification criteria.We planned to treat the patient with high-dose glucocorticoid(GC),followed by cyclophosphamide pulse therapy.After treatment with high-dose GC and an immunosuppressant,imaging studies showed that IHD dilatation had completely resolved.CONCLUSION Prompt diagnosis and appropriate treatment of IgG4-SC are important.Because there is a risk of relapse of IgG4-SC,the GC dose should be gradually reduced,and a maintenance immunosuppressant should be given.展开更多
Background: Rhupus is the rare association of two autoimmune diseases, systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA) diagnosed according to validated ACR criteria. We present the first series of rhu...Background: Rhupus is the rare association of two autoimmune diseases, systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA) diagnosed according to validated ACR criteria. We present the first series of rhupus in Niger. Observation 1: This is a patient suffering from chronic polyarthritis in whom the diagnosis of active seropositive, erosive and deforming RA had been made according to the 1987 ACR criteria and SLE on the presence of 4 criteria according to the 1997 ARA criteria and putting under conventional synthetic DMARDs. The outcome was favorable with a remission achieved after 8 weeks with a disease activity score (DAS) 28 at 2.2. Observation 2: 58-year-old patient with known diabetes type 2 in whom the diagnosis of rhupus was made according to the ACR/EULAR 2010 criteria for RA with a DAS28 of 3.6 and the 2019 LES criteria at 18 points with a SLEDAI score of 6. The evolution was marked by the occurrence of a myocardial infarction with a favorable outcome under medical management. Conclusion: Rhupus is a rare entity that can be complicated by cardiovascular events with the presence of an independent cardiovascular risk factor.展开更多
Rheumatoid arthritis is a chronic multisystem disease of unknown cause. The characteristic feature of RA is persistent inflammatory synovitis. The natural history of disease is such that the early months of the diseas...Rheumatoid arthritis is a chronic multisystem disease of unknown cause. The characteristic feature of RA is persistent inflammatory synovitis. The natural history of disease is such that the early months of the disease are critical period during which reversible joint damage occurs. So early diagnosis of RA and appropriate drug application is the only way to save a patient from this crippling disease. In India, the cost of investigations is a significant factor for most of the patients. Ultrasonography or Power Doppler Ultra Sound (PDUS) has the advantage of being economic in spite of its sensitivity in assessing both inflammatory and destructive changes. The aim of the present study was to evaluate the diagnostic efficiency of PDUS in early rheumatoid arthritis. The study was performed with the patients attending Rheumatology Clinic. A total number of 106 patients of clinically suspected rheumatoid arthritis were studied as per selection criteria. Radiological examinations of hands were done by digital radiography and PDUS in a group of 53 patients, assessment of foot changes by PDUS and Digital Radiography were done in another similar group of 53 patients. Final diagnosis by ACR EULAR-2010 criteria is done for all the patients. The comparative study reveals that synovial vascularity as demonstrated by PDUS is much more effective in diagnosing early rheumatoid arthritis, both in hand and in feet than digital radiograph. PDUS of feet may yield earlier and better findings than hands, which is conventionally used in patients suffering from early rheumatoid arthritis.展开更多
文摘BACKGROUND Immunoglobulin G4-related disease(IgG4-RD)is a multi-system fibroinflammatory disorder that can involve any organ,including the salivary glands,pancreas,and biliary tree.Treatment of immunoglobulin G4-related sclerosing cholangitis(IgG4-SC)is similar to that for IgG4-RD,but progression is irreversible in some cases.We present a case of IgG4-SC in which an immuno-suppressant induced marked clinical and radiologic improvement.CASE SUMMARY A 63-year-old male presented with a prominent itching sensation and wholebody jaundice.He showed obstructive-pattern jaundice,an elevated IgG4 level,and infiltration of a large number of IgG4-positive cells in the ampulla of Vater.The imaging findings of intrahepatic duct(IHD)and common bile duct dilation,an elevated serum IgG4 level,and characteristic histological findings led to diagnosis of IgG4-SC that compatible with the 2019 ACR/EULAR classification criteria.We planned to treat the patient with high-dose glucocorticoid(GC),followed by cyclophosphamide pulse therapy.After treatment with high-dose GC and an immunosuppressant,imaging studies showed that IHD dilatation had completely resolved.CONCLUSION Prompt diagnosis and appropriate treatment of IgG4-SC are important.Because there is a risk of relapse of IgG4-SC,the GC dose should be gradually reduced,and a maintenance immunosuppressant should be given.
文摘Background: Rhupus is the rare association of two autoimmune diseases, systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA) diagnosed according to validated ACR criteria. We present the first series of rhupus in Niger. Observation 1: This is a patient suffering from chronic polyarthritis in whom the diagnosis of active seropositive, erosive and deforming RA had been made according to the 1987 ACR criteria and SLE on the presence of 4 criteria according to the 1997 ARA criteria and putting under conventional synthetic DMARDs. The outcome was favorable with a remission achieved after 8 weeks with a disease activity score (DAS) 28 at 2.2. Observation 2: 58-year-old patient with known diabetes type 2 in whom the diagnosis of rhupus was made according to the ACR/EULAR 2010 criteria for RA with a DAS28 of 3.6 and the 2019 LES criteria at 18 points with a SLEDAI score of 6. The evolution was marked by the occurrence of a myocardial infarction with a favorable outcome under medical management. Conclusion: Rhupus is a rare entity that can be complicated by cardiovascular events with the presence of an independent cardiovascular risk factor.
文摘Rheumatoid arthritis is a chronic multisystem disease of unknown cause. The characteristic feature of RA is persistent inflammatory synovitis. The natural history of disease is such that the early months of the disease are critical period during which reversible joint damage occurs. So early diagnosis of RA and appropriate drug application is the only way to save a patient from this crippling disease. In India, the cost of investigations is a significant factor for most of the patients. Ultrasonography or Power Doppler Ultra Sound (PDUS) has the advantage of being economic in spite of its sensitivity in assessing both inflammatory and destructive changes. The aim of the present study was to evaluate the diagnostic efficiency of PDUS in early rheumatoid arthritis. The study was performed with the patients attending Rheumatology Clinic. A total number of 106 patients of clinically suspected rheumatoid arthritis were studied as per selection criteria. Radiological examinations of hands were done by digital radiography and PDUS in a group of 53 patients, assessment of foot changes by PDUS and Digital Radiography were done in another similar group of 53 patients. Final diagnosis by ACR EULAR-2010 criteria is done for all the patients. The comparative study reveals that synovial vascularity as demonstrated by PDUS is much more effective in diagnosing early rheumatoid arthritis, both in hand and in feet than digital radiograph. PDUS of feet may yield earlier and better findings than hands, which is conventionally used in patients suffering from early rheumatoid arthritis.
