Raynaud’s phenomenon is a symptom complex manifested as intermittent fingertip ischemia caused by cold or other sympathetic drivers.Secondary Raynaud’s phenomenon is often more severe and could even lead to finger u...Raynaud’s phenomenon is a symptom complex manifested as intermittent fingertip ischemia caused by cold or other sympathetic drivers.Secondary Raynaud’s phenomenon is often more severe and could even lead to finger ulceration,making it particularly complicated to treat.We describe a case of severe Raynaud’s phenomenon secondary to subclinical hypothyroidism lasting for more than 6 hours in a 65-year-old woman.The patient was also diagnosed with hypothyroidism,epilepsy,and secondary soft tissue infection of the right middle and ring fingers.After careful multidisciplinary consultation and discussion,the patient received vasodilation,anticoagulation,thyroxine supplementation,stellate ganglion block,hyperbaric oxygen therapy and debridement.The patient responded well to the medication,avoiding amputation or obviously dysfunction.Multidisciplinary team gathering the doctors from different departments proposes appropriate strategies for patients with severe Raynaud’s phenomenon and could improve the prognosis and satisfaction of patient effectively.展开更多
Raynaud’s syndrome (RS) is a vasospastic disorder with potential hereditary predisposition and multiple triggering factors. After the exclusion of external stimuli and local disorders, autoimmunity presents a potenti...Raynaud’s syndrome (RS) is a vasospastic disorder with potential hereditary predisposition and multiple triggering factors. After the exclusion of external stimuli and local disorders, autoimmunity presents a potential intrinsic trigger even in idiopathic RS. We present a 47-year-old woman with severe and progressive RS in both hands and toes for 2 years. She did not have: a) clinical and laboratory evidence of systemic autoimmune diseases;b) prior drug exposure;c) local digits disease. She could not tolerate vasodilators. Since she had elevated C-reactive protein, she received MMF as immunosuppressive therapy. Within 2 weeks, her symptoms improved, and she remained disease-free for 2 years. In conclusion, blocking intrinsic autoimmune triggers with MMF is a safe, practical, and effective short- and long-term treatment for refractory primary RS.展开更多
AIM:To establish the frequency and clinical features of connective tissue diseases(CTDs)in a cohort of Chinese patients with primary biliary cirrhosis(PBC).METHODS:Three-hundred and twenty-two Chinese PBC patients wer...AIM:To establish the frequency and clinical features of connective tissue diseases(CTDs)in a cohort of Chinese patients with primary biliary cirrhosis(PBC).METHODS:Three-hundred and twenty-two Chinese PBC patients were screened for the presence of CTD,and the systemic involvement was assessed.The differences in clinical features and laboratory findings between PBC patients with and without CTD were documented.The diversity of incidence of CTDs in PBC of different countries and areas was discussed.For the comparison of normally distributed data,Student’s t test was used,while non-parametric test(Wilcoxon test)for the non-normally distributed data and 2×2χ2or Fisher’s exact tests for the ratio.RESULTS:One-hundred and fifty(46.6%)PBC patients had one or more CTDs.The most common CTD was Sj gren’s syndrome(SS,121 cases,36.2%).There were nine cases of systemic sclerosis(SSc,2.8%),12of systemic lupus erythematosus(SLE,3.7%),nine of rheumatoid arthritis(RA,2.8%),and 10 of polymyositis(PM,3.1%)in this cohort.Compared to patients with PBC only,the PBC+SS patients were more likely to have fever and elevated erythrocyte sedimentation rate(ESR),higher serum immunoglobulin G(IgG)levels and more frequent rheumatoid factor(RF)and interstitial lung disease(ILD)incidences;PBC+SSc patients had higher frequency of ILD;PBC+SLE patients had lower white blood cell(WBC)count,hemoglobin(Hb),platelet count,γ-glutamyl transpeptidase and immunoglobulin M levels,but higher frequency of renal involvement;PBC+RA patients had lower Hb,higher serum IgG,alkaline phosphatase,faster ESR and a higher ratio of RF positivity;PBC+PM patients had higher WBC count and a tendency towards myocardial involvement.CONCLUSION:Besides the common liver manifestation of PBC,systemic involvement and overlaps with other CTDs are not infrequent in Chinese patients.When overlapping with other CTDs,PBC patients manifested some special clinical and laboratory features which may have effect on the prognosis.展开更多
文摘Raynaud’s phenomenon is a symptom complex manifested as intermittent fingertip ischemia caused by cold or other sympathetic drivers.Secondary Raynaud’s phenomenon is often more severe and could even lead to finger ulceration,making it particularly complicated to treat.We describe a case of severe Raynaud’s phenomenon secondary to subclinical hypothyroidism lasting for more than 6 hours in a 65-year-old woman.The patient was also diagnosed with hypothyroidism,epilepsy,and secondary soft tissue infection of the right middle and ring fingers.After careful multidisciplinary consultation and discussion,the patient received vasodilation,anticoagulation,thyroxine supplementation,stellate ganglion block,hyperbaric oxygen therapy and debridement.The patient responded well to the medication,avoiding amputation or obviously dysfunction.Multidisciplinary team gathering the doctors from different departments proposes appropriate strategies for patients with severe Raynaud’s phenomenon and could improve the prognosis and satisfaction of patient effectively.
文摘Raynaud’s syndrome (RS) is a vasospastic disorder with potential hereditary predisposition and multiple triggering factors. After the exclusion of external stimuli and local disorders, autoimmunity presents a potential intrinsic trigger even in idiopathic RS. We present a 47-year-old woman with severe and progressive RS in both hands and toes for 2 years. She did not have: a) clinical and laboratory evidence of systemic autoimmune diseases;b) prior drug exposure;c) local digits disease. She could not tolerate vasodilators. Since she had elevated C-reactive protein, she received MMF as immunosuppressive therapy. Within 2 weeks, her symptoms improved, and she remained disease-free for 2 years. In conclusion, blocking intrinsic autoimmune triggers with MMF is a safe, practical, and effective short- and long-term treatment for refractory primary RS.
基金Supported by Grants from the Research Special Fund for Public Welfare Industry of Health,No.201202004the National Major Scientific and Technological Special Project for"Significant New Drugs Development",No.2012ZX09303006-002the National High Technology Research and Development Program of China,No.2011AA020111
文摘AIM:To establish the frequency and clinical features of connective tissue diseases(CTDs)in a cohort of Chinese patients with primary biliary cirrhosis(PBC).METHODS:Three-hundred and twenty-two Chinese PBC patients were screened for the presence of CTD,and the systemic involvement was assessed.The differences in clinical features and laboratory findings between PBC patients with and without CTD were documented.The diversity of incidence of CTDs in PBC of different countries and areas was discussed.For the comparison of normally distributed data,Student’s t test was used,while non-parametric test(Wilcoxon test)for the non-normally distributed data and 2×2χ2or Fisher’s exact tests for the ratio.RESULTS:One-hundred and fifty(46.6%)PBC patients had one or more CTDs.The most common CTD was Sj gren’s syndrome(SS,121 cases,36.2%).There were nine cases of systemic sclerosis(SSc,2.8%),12of systemic lupus erythematosus(SLE,3.7%),nine of rheumatoid arthritis(RA,2.8%),and 10 of polymyositis(PM,3.1%)in this cohort.Compared to patients with PBC only,the PBC+SS patients were more likely to have fever and elevated erythrocyte sedimentation rate(ESR),higher serum immunoglobulin G(IgG)levels and more frequent rheumatoid factor(RF)and interstitial lung disease(ILD)incidences;PBC+SSc patients had higher frequency of ILD;PBC+SLE patients had lower white blood cell(WBC)count,hemoglobin(Hb),platelet count,γ-glutamyl transpeptidase and immunoglobulin M levels,but higher frequency of renal involvement;PBC+RA patients had lower Hb,higher serum IgG,alkaline phosphatase,faster ESR and a higher ratio of RF positivity;PBC+PM patients had higher WBC count and a tendency towards myocardial involvement.CONCLUSION:Besides the common liver manifestation of PBC,systemic involvement and overlaps with other CTDs are not infrequent in Chinese patients.When overlapping with other CTDs,PBC patients manifested some special clinical and laboratory features which may have effect on the prognosis.
