BACKGROUND This study presents a case of rapidly developing respiratory failure due to antisynthetase syndrome(AS)following coronavirus disease 2019(COVID-19)in a 33-year-old man diagnosed with Klinefelter syndrome(KS...BACKGROUND This study presents a case of rapidly developing respiratory failure due to antisynthetase syndrome(AS)following coronavirus disease 2019(COVID-19)in a 33-year-old man diagnosed with Klinefelter syndrome(KS).CASE SUMMARY A 33-year-old man with a diagnosis of KS was admitted to the Department of Pulmonary and Critical Care Medicine of a tertiary hospital in China for fever and shortness of breath 2 wk after the onset of COVID-19.Computed tomography of both lungs revealed diffuse multiple patchy heightened shadows in both lungs,accompanied by signs of partial bronchial inflation.Metagenomic next-generation sequencing of the bronchoalveolar lavage fluid suggested absence of pathogen.A biopsy specimen revealed organizing pneumonia with alveolar septal thickening.Additionally,extensive auto-antibody tests showed strong positivity for anti-SSA,anti-SSB,anti-Jo-1,and anti-Ro-52.Following multidisciplinary discussions,the patient received a final diagnosis of AS,leading to rapidly progressing respiratory failure.CONCLUSION This study underscores the clinical progression of AS-associated interstitial lung disease subsequent to viral infections such as COVID-19 in patients diagnosed with KS.展开更多
BACKGROUND Polyarthritis is the most frequent clinical manifestation in antisynthetase syndrome(ASS)forms of idiopathic inflammatory myositis and may be misdiagnosed as rheumatoid arthritis(RA),particularly in patient...BACKGROUND Polyarthritis is the most frequent clinical manifestation in antisynthetase syndrome(ASS)forms of idiopathic inflammatory myositis and may be misdiagnosed as rheumatoid arthritis(RA),particularly in patients with seronegative RA(SNRA).It is unclear whether there is an overlap between ASS and RA,or if ASS sometimes mimics RA.Pulmonary hypertension(PAH)is common in connective tissue diseases(CTDs).However,published reports on CTD-PAH do not include overlapping CTDs,and its incidence and impact on patient prognosis are unclear.CASE SUMMARY We report the case of a 63-year-old woman who presented with a 3-mo history of symptom aggravation of recurrent symmetrical joint swelling and pain that had persisted for over 10 years.The patient was diagnosed with RA and interstitial lung disease.The patient repeatedly presented to the hospital’s respiratory and rhe-umatology departments with arthralgia,plus shortness of breath after activity.Relevant tests indicated that anti-CCP and RF remained negative,while anti-J0-1 and anti-Ro-52 were strongly positive.It was not until recently that we recognized that this could be an unusual case of SNRA with concurrent ASS.Joint pain was relieved after regular anti-rheumatic treatment.Chest computed tomography scans showed that pulmonary interstitial changes did not progress significantly over several years;however,they showed gradual widening of the pulmonary artery,and cardiac ultrasound indicated elevated pulmonary artery systolic pressure.The prescribed treatment of PAH was not effective in improving shortness of breath.CONCLUSION Overlap of RA and ASS may be missed.Further research is necessary to facilitate early diagnosis,effective evaluation,and prognosis.展开更多
BACKGROUND Antisynthetase syndrome(ASS)is characterized by the presence of antisynthetase antibodies coupled with clinical findings such as fever,polymyositis-dermatomyositis and interstitial lung disease.It is,howeve...BACKGROUND Antisynthetase syndrome(ASS)is characterized by the presence of antisynthetase antibodies coupled with clinical findings such as fever,polymyositis-dermatomyositis and interstitial lung disease.It is,however,rare to observe ASS association with B cell lymphoma presenting severe pneumonia as the first clinical manifestation.CASE SUMMARY We evaluated a 59-year-old male patient who presented with cough with sputum,shortness of breath and fever for 13 d.A chest computed tomography radiograph revealed bilateral diffuse ground-glass infiltrates in both upper fields,left lingual lobe and right middle lobe.Initially,the patient was diagnosed with severe community-acquired pneumonia and respiratory failure.He was empirically treated with broad-spectrum antibiotics,without improvement.Further analysis showed an ASS panel with anti-PL7 antibodies.Besides,electromyography evaluation demonstrated a manifestation of myogenic damage,while deltoid muscle biopsy showed irregular muscle fiber bundles especially abnormal lymphocyte infiltration.In addition,bone marrow biopsy revealed high invasive B cell lymphoma.Thus,the patient was diagnosed with a relatively rare anti–PL7 antibody positive ASS associated with B cell lymphoma.CONCLUSION This case highlights that rapidly progressive lung lesions and acute hypoxemic respiratory failure associated with heliotrope rash and extremely high lactate dehydrogenase level should be considered as the characteristics of non-infectious diseases,especially ASS and B cell lymphoma.展开更多
Introduction: Antisynthetase syndrome is an original entity and rare autoimmune myositis and systemic disease, characterized clinically by a wide spectrum of clinical manifestations and the presence of autoantibodies ...Introduction: Antisynthetase syndrome is an original entity and rare autoimmune myositis and systemic disease, characterized clinically by a wide spectrum of clinical manifestations and the presence of autoantibodies directed against aminoacyl RNAt synthetases. We describe this disease in 03 Senegalese patients. Observations: The first patient was a 49-years-old black woman who was referred in our department after 06-months of follow-up for a misdiagnosis of tuberculosis. The clinical examination revealed polyarthritis, muscle weakness, chronic cough with crackling rales at the pulmonary bases, Raynaud phenomenon and dry syndrome. The second patient, a 21-years-old black woman, had polyarthritis and a progressive muscle weakness. The clinical examination showed also cutaneous signs including an erythema on the dorsal part of the fingers and the presence of the heliotrope erythema on the eyes. The last patient was a 52 years-old black woman. His clinical examination showed polyarthritis, muscle weakness and an appearance of mechanics’ hands. The creatinine phosphokinase was at 6.26 × N, 40.3 × N and 33.64 × N respectively in our patients. The chest computer tomography revealed an interstitial lung disease with a pattern of non-specific interstitial pneumonia in all three patients. The autoantibodies anti-Jo1 was also positive in all patients. The diagnosis of antisynthetase syndrome was retained with an overlap of antisynthetase and Sj?gren’s syndrome in the first observation. The evolution was favourable in our 03 observations with a therapeutic combination including Prednisone-Azathioprine and Kinesitherapy. Conclusion: Antisynthetase syndrome has been exceptionally reported in sub-Saharan Africa. It must be particularly mentioned in front of the triad: myositis, arthritis and interstitial lung disease. The identification of an auto-antibody directed against RNA t synthetases, particularly anti-Jo1, is essential for its diagnosis. Prognosis is related to interstitial lung involvement. The evolution has been favourable in our patients receiving Glucorticoid-Azathioprine combination therapy.展开更多
基金Supported by the Natural Science Foundation of Jiangxi Province,No.20202BAB206002 and No.20224BAB216084.
文摘BACKGROUND This study presents a case of rapidly developing respiratory failure due to antisynthetase syndrome(AS)following coronavirus disease 2019(COVID-19)in a 33-year-old man diagnosed with Klinefelter syndrome(KS).CASE SUMMARY A 33-year-old man with a diagnosis of KS was admitted to the Department of Pulmonary and Critical Care Medicine of a tertiary hospital in China for fever and shortness of breath 2 wk after the onset of COVID-19.Computed tomography of both lungs revealed diffuse multiple patchy heightened shadows in both lungs,accompanied by signs of partial bronchial inflation.Metagenomic next-generation sequencing of the bronchoalveolar lavage fluid suggested absence of pathogen.A biopsy specimen revealed organizing pneumonia with alveolar septal thickening.Additionally,extensive auto-antibody tests showed strong positivity for anti-SSA,anti-SSB,anti-Jo-1,and anti-Ro-52.Following multidisciplinary discussions,the patient received a final diagnosis of AS,leading to rapidly progressing respiratory failure.CONCLUSION This study underscores the clinical progression of AS-associated interstitial lung disease subsequent to viral infections such as COVID-19 in patients diagnosed with KS.
基金Supported by the Natural Science Foundation of China,No.82060010。
文摘BACKGROUND Polyarthritis is the most frequent clinical manifestation in antisynthetase syndrome(ASS)forms of idiopathic inflammatory myositis and may be misdiagnosed as rheumatoid arthritis(RA),particularly in patients with seronegative RA(SNRA).It is unclear whether there is an overlap between ASS and RA,or if ASS sometimes mimics RA.Pulmonary hypertension(PAH)is common in connective tissue diseases(CTDs).However,published reports on CTD-PAH do not include overlapping CTDs,and its incidence and impact on patient prognosis are unclear.CASE SUMMARY We report the case of a 63-year-old woman who presented with a 3-mo history of symptom aggravation of recurrent symmetrical joint swelling and pain that had persisted for over 10 years.The patient was diagnosed with RA and interstitial lung disease.The patient repeatedly presented to the hospital’s respiratory and rhe-umatology departments with arthralgia,plus shortness of breath after activity.Relevant tests indicated that anti-CCP and RF remained negative,while anti-J0-1 and anti-Ro-52 were strongly positive.It was not until recently that we recognized that this could be an unusual case of SNRA with concurrent ASS.Joint pain was relieved after regular anti-rheumatic treatment.Chest computed tomography scans showed that pulmonary interstitial changes did not progress significantly over several years;however,they showed gradual widening of the pulmonary artery,and cardiac ultrasound indicated elevated pulmonary artery systolic pressure.The prescribed treatment of PAH was not effective in improving shortness of breath.CONCLUSION Overlap of RA and ASS may be missed.Further research is necessary to facilitate early diagnosis,effective evaluation,and prognosis.
基金Supported by National Natural Science Foundation of China,No.81900020Natural Science Foundation of Zhejiang Province,China,No.LQ19H160020.
文摘BACKGROUND Antisynthetase syndrome(ASS)is characterized by the presence of antisynthetase antibodies coupled with clinical findings such as fever,polymyositis-dermatomyositis and interstitial lung disease.It is,however,rare to observe ASS association with B cell lymphoma presenting severe pneumonia as the first clinical manifestation.CASE SUMMARY We evaluated a 59-year-old male patient who presented with cough with sputum,shortness of breath and fever for 13 d.A chest computed tomography radiograph revealed bilateral diffuse ground-glass infiltrates in both upper fields,left lingual lobe and right middle lobe.Initially,the patient was diagnosed with severe community-acquired pneumonia and respiratory failure.He was empirically treated with broad-spectrum antibiotics,without improvement.Further analysis showed an ASS panel with anti-PL7 antibodies.Besides,electromyography evaluation demonstrated a manifestation of myogenic damage,while deltoid muscle biopsy showed irregular muscle fiber bundles especially abnormal lymphocyte infiltration.In addition,bone marrow biopsy revealed high invasive B cell lymphoma.Thus,the patient was diagnosed with a relatively rare anti–PL7 antibody positive ASS associated with B cell lymphoma.CONCLUSION This case highlights that rapidly progressive lung lesions and acute hypoxemic respiratory failure associated with heliotrope rash and extremely high lactate dehydrogenase level should be considered as the characteristics of non-infectious diseases,especially ASS and B cell lymphoma.
文摘Introduction: Antisynthetase syndrome is an original entity and rare autoimmune myositis and systemic disease, characterized clinically by a wide spectrum of clinical manifestations and the presence of autoantibodies directed against aminoacyl RNAt synthetases. We describe this disease in 03 Senegalese patients. Observations: The first patient was a 49-years-old black woman who was referred in our department after 06-months of follow-up for a misdiagnosis of tuberculosis. The clinical examination revealed polyarthritis, muscle weakness, chronic cough with crackling rales at the pulmonary bases, Raynaud phenomenon and dry syndrome. The second patient, a 21-years-old black woman, had polyarthritis and a progressive muscle weakness. The clinical examination showed also cutaneous signs including an erythema on the dorsal part of the fingers and the presence of the heliotrope erythema on the eyes. The last patient was a 52 years-old black woman. His clinical examination showed polyarthritis, muscle weakness and an appearance of mechanics’ hands. The creatinine phosphokinase was at 6.26 × N, 40.3 × N and 33.64 × N respectively in our patients. The chest computer tomography revealed an interstitial lung disease with a pattern of non-specific interstitial pneumonia in all three patients. The autoantibodies anti-Jo1 was also positive in all patients. The diagnosis of antisynthetase syndrome was retained with an overlap of antisynthetase and Sj?gren’s syndrome in the first observation. The evolution was favourable in our 03 observations with a therapeutic combination including Prednisone-Azathioprine and Kinesitherapy. Conclusion: Antisynthetase syndrome has been exceptionally reported in sub-Saharan Africa. It must be particularly mentioned in front of the triad: myositis, arthritis and interstitial lung disease. The identification of an auto-antibody directed against RNA t synthetases, particularly anti-Jo1, is essential for its diagnosis. Prognosis is related to interstitial lung involvement. The evolution has been favourable in our patients receiving Glucorticoid-Azathioprine combination therapy.
