Pub Date : 1900-01-01DOI: 10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2046
Ria E Gripaldo, L. Weerheim, U. Specks, M. Baqir
{"title":"Treatment of Idiopathic Pulmonary Hemosiderosis with Rituximab","authors":"Ria E Gripaldo, L. Weerheim, U. Specks, M. Baqir","doi":"10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2046","DOIUrl":"https://doi.org/10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2046","url":null,"abstract":"","PeriodicalId":114116,"journal":{"name":"TP33. TP033 CASE REPORTS IN AUTOIMMUNE LUNG DISEASE","volume":"49 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"1900-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"123805763","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 1900-01-01DOI: 10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2062
S. Chalmers, C. Thomas
{"title":"Pulmonary Nodules in a Young Female with Ulcers, Skin Nodules, and Thrombosis","authors":"S. Chalmers, C. Thomas","doi":"10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2062","DOIUrl":"https://doi.org/10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2062","url":null,"abstract":"","PeriodicalId":114116,"journal":{"name":"TP33. TP033 CASE REPORTS IN AUTOIMMUNE LUNG DISEASE","volume":"27 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"1900-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"133939397","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 1900-01-01DOI: 10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2040
M. Thomas, P. Deshpande, L. Tran, R. Sampath
{"title":"Rapidly Progressive Interstitial Lung Disease Associated with Anti-MDA5 Dermatomyositis","authors":"M. Thomas, P. Deshpande, L. Tran, R. Sampath","doi":"10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2040","DOIUrl":"https://doi.org/10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2040","url":null,"abstract":"","PeriodicalId":114116,"journal":{"name":"TP33. TP033 CASE REPORTS IN AUTOIMMUNE LUNG DISEASE","volume":"105 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"1900-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"124067931","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 1900-01-01DOI: 10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2042
P. Ho, A. J. Rosenbaum, N. Salam, L. Lande, S. A. Gregory
Diffuse alveolar hemorrhage (DAH) is a life-threatening condition associated with various systemic diseases. Evaluation includes determining an underlying etiology. Isolated pauci-immune pulmonary capillaritis (IPIPC) without clinical, serological, or histological evidence of an underlying systemic process is an extremely rare cause of DAH. In limited case series, treatment is similar to treatment of DAH secondary to vasculitis or autoimmune disease and consists of immunosuppression. Prognosis is generally favorable. We present a case of a healthy male with IPIPC who, despite treatment, had persistent and ultimately fatal DAH. A 64-year-old male presented with mild dyspnea and scant hemoptysis. Review of systems was unremarkable. There was no history of travel or exposures. Hemoglobin was 12.0 g/dL, creatinine was 0.9 mg/dL, and urinalysis was normal. CT scan of the chest showed bilateral patchy ground glass opacities. Nasal swab for SARS-CoV-2 was negative. Bronchoscopy confirmed diffuse alveolar hemorrhage. He was started on methylprednisolone 1g daily for 3 days, followed by 1 mg/kg daily. Coagulation studies, ANA, RF, ANCA, and anti-GBM antibodies were negative. ESR was normal, but CRP was elevated at 29 mg/L. His echocardiogram was normal. Six days after bronchoscopy, he underwent a surgical lung biopsy. Pathology showed mostly bland alveolar hemorrhage with scattered areas of neutrophilic infiltration of the alveolar walls. Immunostaining was negative. Cyclophosphamide 2 mg/kg was initiated on day 14. The patient was maintained on nasal oxygen but had persistent slow alveolar bleeding. On day 22, plasmapheresis was initiated. Despite after 5 treatments of plasmapheresis, bleeding persisted. Cyclophosphamide was discontinued and rituximab was initiated. Over the next four weeks, bleeding accelerated with worsening hypoxemia, which ultimately led to intubation. The patient spent two weeks on the ventilator with need for daily blood transfusions. Gas exchange worsened with progressive hypoxemia and hypercapnia. Nine weeks after his initial presentation, the patient expired after family requested for palliative extubation. Autopsy showed hemorrhagic lungs without evidence of infection or malignancy. Isolated pauci-immune pulmonary capillaritis is rare. Since it was first described in 1995, there have been a limited number of cases described in literature. Current treatment recommendations are similar to treatment of DAH due to vasculitis, which include corticosteroids combined with cyclophosphamide or rituximab. Prognosis is generally favorable. Progressive and relentless hemorrhage as described in this case is unusual.
{"title":"A Case of Idiopathic Pauci-Immune Pulmonary Capillaritis: A Rare Form of Diffuse Alveolar Hemorrhage","authors":"P. Ho, A. J. Rosenbaum, N. Salam, L. Lande, S. A. Gregory","doi":"10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2042","DOIUrl":"https://doi.org/10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2042","url":null,"abstract":"Diffuse alveolar hemorrhage (DAH) is a life-threatening condition associated with various systemic diseases. Evaluation includes determining an underlying etiology. Isolated pauci-immune pulmonary capillaritis (IPIPC) without clinical, serological, or histological evidence of an underlying systemic process is an extremely rare cause of DAH. In limited case series, treatment is similar to treatment of DAH secondary to vasculitis or autoimmune disease and consists of immunosuppression. Prognosis is generally favorable. We present a case of a healthy male with IPIPC who, despite treatment, had persistent and ultimately fatal DAH. A 64-year-old male presented with mild dyspnea and scant hemoptysis. Review of systems was unremarkable. There was no history of travel or exposures. Hemoglobin was 12.0 g/dL, creatinine was 0.9 mg/dL, and urinalysis was normal. CT scan of the chest showed bilateral patchy ground glass opacities. Nasal swab for SARS-CoV-2 was negative. Bronchoscopy confirmed diffuse alveolar hemorrhage. He was started on methylprednisolone 1g daily for 3 days, followed by 1 mg/kg daily. Coagulation studies, ANA, RF, ANCA, and anti-GBM antibodies were negative. ESR was normal, but CRP was elevated at 29 mg/L. His echocardiogram was normal. Six days after bronchoscopy, he underwent a surgical lung biopsy. Pathology showed mostly bland alveolar hemorrhage with scattered areas of neutrophilic infiltration of the alveolar walls. Immunostaining was negative. Cyclophosphamide 2 mg/kg was initiated on day 14. The patient was maintained on nasal oxygen but had persistent slow alveolar bleeding. On day 22, plasmapheresis was initiated. Despite after 5 treatments of plasmapheresis, bleeding persisted. Cyclophosphamide was discontinued and rituximab was initiated. Over the next four weeks, bleeding accelerated with worsening hypoxemia, which ultimately led to intubation. The patient spent two weeks on the ventilator with need for daily blood transfusions. Gas exchange worsened with progressive hypoxemia and hypercapnia. Nine weeks after his initial presentation, the patient expired after family requested for palliative extubation. Autopsy showed hemorrhagic lungs without evidence of infection or malignancy. Isolated pauci-immune pulmonary capillaritis is rare. Since it was first described in 1995, there have been a limited number of cases described in literature. Current treatment recommendations are similar to treatment of DAH due to vasculitis, which include corticosteroids combined with cyclophosphamide or rituximab. Prognosis is generally favorable. Progressive and relentless hemorrhage as described in this case is unusual.","PeriodicalId":114116,"journal":{"name":"TP33. TP033 CASE REPORTS IN AUTOIMMUNE LUNG DISEASE","volume":"15 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"1900-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"126125078","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 1900-01-01DOI: 10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2045
Z. Cawasji, B. A. Jaumally, R. Witkov, R. Mathew
{"title":"Antiphospholipid Syndrome: A Rare Cause of Diffuse Alveolar Hemorrhage","authors":"Z. Cawasji, B. A. Jaumally, R. Witkov, R. Mathew","doi":"10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2045","DOIUrl":"https://doi.org/10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2045","url":null,"abstract":"","PeriodicalId":114116,"journal":{"name":"TP33. TP033 CASE REPORTS IN AUTOIMMUNE LUNG DISEASE","volume":"13 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"1900-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"124881074","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 1900-01-01DOI: 10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2060
A. Kudirka, A. Abdul Hameed, K. Thavarajah, K. Maksimowicz-McKinnon, A. Abu Sayf
{"title":"Interstitial Lung Disease in Patients with Anti-Ro52 Antibody: A Case Report","authors":"A. Kudirka, A. Abdul Hameed, K. Thavarajah, K. Maksimowicz-McKinnon, A. Abu Sayf","doi":"10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2060","DOIUrl":"https://doi.org/10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2060","url":null,"abstract":"","PeriodicalId":114116,"journal":{"name":"TP33. TP033 CASE REPORTS IN AUTOIMMUNE LUNG DISEASE","volume":"34 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"1900-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"116698271","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 1900-01-01DOI: 10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2057
G. Mudigonda, D. Song, R. Hansra, P. Charoenpong
Introduction. Anti-Synthetase Syndromes and IgG4-related diseases have rarely been seen together. We present a 38-year-old female with connective tissue disease-associated interstitial lung disease from Anti-Synthetase syndrome who eventually develops autoimmune pancreatitis from IgG4-related disease. Case Presentation. A 38-year-old African American female with facial rash, joint swelling, and morning stiffness was seen to have pulmonary fibrosis on CXR and CT chest. Subsequent bronchoscopy was inconclusive. She was treated for presumed mixed Connected Tissue Disease (CTD) and associated pulmonary fibrosis with Mycophenolate, Hydroxychloroquine, and Prednisone. Several months later, she had shortness of breath and was diagnosed with COVID-19. Her mycophenolate was held while she recovered with supplemental oxygen, but has required 2 to 3 liters of supplemental oxygen chronically since her COVID diagnosis. A repeat bronchoscopy with transbronchial and cryobiopsies was suggestive of Cryptogenic Organizing Pneumonia (COP) and negative for malignancy. At that time, blood work revealed positive melanoma differentiation-associated (MDA) protein 5 antibodies, high antinuclear antibodies (ANA) titers, positive anti-Sjögren's-syndrome antigen A (SSA) autoantibodies, weakly positive PL-12, and negative Jo-1. Additionally, the patient had elevated concentrations of IgG4. A repeat CT revealed fatty infiltration of the pancreas. Her MDA positivity qualified the patient to begin Rituximab infusions weekly for four weeks. Her shortness of breath continued to improve on her previously stated treatments. However, she presented to a local emergency department for acute abdominal pain. She was diagnosed with acute pancreatitis and improved after receiving intravenous fluids. Discussion. This patient has connective tissue disease associated with interstitial lung disease from Anti-Synthetase syndrome. She has positive anti-MDA-5 antibodies and cryobiopsy diagnosed COP, a known finding in Anti-Synthetase syndrome. It is also possible that this patient has overlapping IgG4-related disease, correlated by elevated concentrations of IgG4 and autoimmune pancreatitis. This overlap is not well studied, considering that the pathophysiology of Anti-Synthetase syndrome involves autoimmunity towards aminoacyl-tRNA synthetase while IgG4-related disease pathophysiology is not well known and could involve abnormal activation of CD4-positive T cells. Interestingly, this patient had fatty infiltration of her pancreas found on imaging before she was symptomatic for autoimmune pancreatitis. It is possible that both disease processes were not only active simultaneously but also complicated the progression of one another. Clinicians should consider IgG4-related diseases in patients with Anti-Synthetase Syndrome as it might explain a patient's clinical picture and even change management.
{"title":"An Overlap of Two Pathologies: Anti-Synthetase and IgG4-Related Disease","authors":"G. Mudigonda, D. Song, R. Hansra, P. Charoenpong","doi":"10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2057","DOIUrl":"https://doi.org/10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2057","url":null,"abstract":"Introduction. Anti-Synthetase Syndromes and IgG4-related diseases have rarely been seen together. We present a 38-year-old female with connective tissue disease-associated interstitial lung disease from Anti-Synthetase syndrome who eventually develops autoimmune pancreatitis from IgG4-related disease. Case Presentation. A 38-year-old African American female with facial rash, joint swelling, and morning stiffness was seen to have pulmonary fibrosis on CXR and CT chest. Subsequent bronchoscopy was inconclusive. She was treated for presumed mixed Connected Tissue Disease (CTD) and associated pulmonary fibrosis with Mycophenolate, Hydroxychloroquine, and Prednisone. Several months later, she had shortness of breath and was diagnosed with COVID-19. Her mycophenolate was held while she recovered with supplemental oxygen, but has required 2 to 3 liters of supplemental oxygen chronically since her COVID diagnosis. A repeat bronchoscopy with transbronchial and cryobiopsies was suggestive of Cryptogenic Organizing Pneumonia (COP) and negative for malignancy. At that time, blood work revealed positive melanoma differentiation-associated (MDA) protein 5 antibodies, high antinuclear antibodies (ANA) titers, positive anti-Sjögren's-syndrome antigen A (SSA) autoantibodies, weakly positive PL-12, and negative Jo-1. Additionally, the patient had elevated concentrations of IgG4. A repeat CT revealed fatty infiltration of the pancreas. Her MDA positivity qualified the patient to begin Rituximab infusions weekly for four weeks. Her shortness of breath continued to improve on her previously stated treatments. However, she presented to a local emergency department for acute abdominal pain. She was diagnosed with acute pancreatitis and improved after receiving intravenous fluids. Discussion. This patient has connective tissue disease associated with interstitial lung disease from Anti-Synthetase syndrome. She has positive anti-MDA-5 antibodies and cryobiopsy diagnosed COP, a known finding in Anti-Synthetase syndrome. It is also possible that this patient has overlapping IgG4-related disease, correlated by elevated concentrations of IgG4 and autoimmune pancreatitis. This overlap is not well studied, considering that the pathophysiology of Anti-Synthetase syndrome involves autoimmunity towards aminoacyl-tRNA synthetase while IgG4-related disease pathophysiology is not well known and could involve abnormal activation of CD4-positive T cells. Interestingly, this patient had fatty infiltration of her pancreas found on imaging before she was symptomatic for autoimmune pancreatitis. It is possible that both disease processes were not only active simultaneously but also complicated the progression of one another. Clinicians should consider IgG4-related diseases in patients with Anti-Synthetase Syndrome as it might explain a patient's clinical picture and even change management.","PeriodicalId":114116,"journal":{"name":"TP33. TP033 CASE REPORTS IN AUTOIMMUNE LUNG DISEASE","volume":"89 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"1900-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"125699082","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 1900-01-01DOI: 10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2048
Y. Subat, V. Iyer
{"title":"Linear Obstructive Flow Volume Curve in Relapsing Polychondritis","authors":"Y. Subat, V. Iyer","doi":"10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2048","DOIUrl":"https://doi.org/10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2048","url":null,"abstract":"","PeriodicalId":114116,"journal":{"name":"TP33. TP033 CASE REPORTS IN AUTOIMMUNE LUNG DISEASE","volume":"8 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"1900-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"124756735","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 1900-01-01DOI: 10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2049
E. Alatassi, A. Mahmoud, A. Mohamed, A. Gomaa, I. Ismail-Sayed
{"title":"Unexplained Lactic Acidosis and Digital Cyanosis in a Patient with Acute Myeloid Leukemia and Pancytopenia","authors":"E. Alatassi, A. Mahmoud, A. Mohamed, A. Gomaa, I. Ismail-Sayed","doi":"10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2049","DOIUrl":"https://doi.org/10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2049","url":null,"abstract":"","PeriodicalId":114116,"journal":{"name":"TP33. TP033 CASE REPORTS IN AUTOIMMUNE LUNG DISEASE","volume":"468 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"1900-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"134320716","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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