Pub Date : 1900-01-01DOI: 10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2059
C. E. Jordan, K.S. Reyes-Caballero, M. Samson, J. Lichauco
Introduction: About 15% of patients with interstitial lung disease (ILD) have an underlying systemic rheumatic disease and among them, approximately 2-5% have Systemic Lupus Erythematosus (SLE). Etiology is still unknown but current hypotheses of pathogenesis include inflammatory response to environment pathogens causing influx of inflammatory cells to interstitial and alveolar spaces leading to alveolar epithelial damage. ILD can occur anytime during the course of SLE but on average it develops in patients with disease duration of 10 years and symptoms are non-specific. Usual treatments include systemic steroids, mycophenolate mofetil and cyclophosphamide. Case: This is a case of a 54 year-old female, Filipino with one-month history of productive cough accompanied by episodes of undocumented fever and dyspnea on exertion. Work-ups for COVID-19 including RT-PCR test were done and yielded negative results. Further work-ups were done including highresolution computed tomography (HRCT) of the chest which showed reticular opacities, honeycombing and traction bronchiectasis in both lungs predominantly in lower lobes, pleural thickening and pericardial effusion. Parenchymal findings were consistent with interstitial lung disease. Pulmonary function test showed moderate restrictive ventilator defect with reduced DLCO. ANA and anti-dsDNA results were 1:640 speckled pattern and 41.2 respectively and a score of 13 using EULAR Criteria for SLE. She was given Methylprednisolone 65 mg IV once daily, which she did not tolerate. There was worsening of dyspnea and desaturation, hence she was intubated. Treatment for SLE-ILD was shifted to Intravenous Immunoglobulin (IVIg) 25 g and she was able to receive total of five doses. She was extubated after 11 days and was discharged on the 37th hospital day. Discussion: Diagnosis of SLE-ILD is generally clinical, based on presence of extrapulmonary and serologic evidence of SLE combined with HRCT findings confirming ILD and exclusion of other potential causes. Aside from the usual treatment using systemic steroids, severe cases can be managed using Rituximab while refractory cases are managed with IVIg and plasmapheresis. To date, there are no available practice guidelines for the management of SLE-ILD. The rest of the therapeutic approaches have been extrapolated from those utilized in systemic sclerosis due to the similarity of interstitial involvement. Literatures documenting success of IVIg in management of SLE-ILD are also limited;hence this case was presented to document successful response to IVIg treatment in a patient diagnosed with SLE-ILD.
{"title":"Case of 54 Year-Old Patient with Systemic Lupus Erythematosus-Interstitial Lung Disease Managed with Intravenous Immunoglobulin","authors":"C. E. Jordan, K.S. Reyes-Caballero, M. Samson, J. Lichauco","doi":"10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2059","DOIUrl":"https://doi.org/10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2059","url":null,"abstract":"Introduction: About 15% of patients with interstitial lung disease (ILD) have an underlying systemic rheumatic disease and among them, approximately 2-5% have Systemic Lupus Erythematosus (SLE). Etiology is still unknown but current hypotheses of pathogenesis include inflammatory response to environment pathogens causing influx of inflammatory cells to interstitial and alveolar spaces leading to alveolar epithelial damage. ILD can occur anytime during the course of SLE but on average it develops in patients with disease duration of 10 years and symptoms are non-specific. Usual treatments include systemic steroids, mycophenolate mofetil and cyclophosphamide. Case: This is a case of a 54 year-old female, Filipino with one-month history of productive cough accompanied by episodes of undocumented fever and dyspnea on exertion. Work-ups for COVID-19 including RT-PCR test were done and yielded negative results. Further work-ups were done including highresolution computed tomography (HRCT) of the chest which showed reticular opacities, honeycombing and traction bronchiectasis in both lungs predominantly in lower lobes, pleural thickening and pericardial effusion. Parenchymal findings were consistent with interstitial lung disease. Pulmonary function test showed moderate restrictive ventilator defect with reduced DLCO. ANA and anti-dsDNA results were 1:640 speckled pattern and 41.2 respectively and a score of 13 using EULAR Criteria for SLE. She was given Methylprednisolone 65 mg IV once daily, which she did not tolerate. There was worsening of dyspnea and desaturation, hence she was intubated. Treatment for SLE-ILD was shifted to Intravenous Immunoglobulin (IVIg) 25 g and she was able to receive total of five doses. She was extubated after 11 days and was discharged on the 37th hospital day. Discussion: Diagnosis of SLE-ILD is generally clinical, based on presence of extrapulmonary and serologic evidence of SLE combined with HRCT findings confirming ILD and exclusion of other potential causes. Aside from the usual treatment using systemic steroids, severe cases can be managed using Rituximab while refractory cases are managed with IVIg and plasmapheresis. To date, there are no available practice guidelines for the management of SLE-ILD. The rest of the therapeutic approaches have been extrapolated from those utilized in systemic sclerosis due to the similarity of interstitial involvement. Literatures documenting success of IVIg in management of SLE-ILD are also limited;hence this case was presented to document successful response to IVIg treatment in a patient diagnosed with SLE-ILD.","PeriodicalId":114116,"journal":{"name":"TP33. TP033 CASE REPORTS IN AUTOIMMUNE LUNG DISEASE","volume":"24 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":"115076321","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.a2064
F. Mfone, O. Enilari, V. Pathak
Introduction: Interstitial Lung Disease (ILD) is the heterogenous group of disease that share similar clinical, radiological and physiological findings and hence mostly grouped together. Radiologically, they present with varied findings which includes ground glass opacity, reticulations, honey combing, septal thickenings or the combination of these findings. Some specific combination could lead to the diagnosis of a particular ILD provided radiological findings match the clinical presentations. We present a patient who came with chronic cough and shortness of breath and CT chest showed anterior mediastinal mass with bilateral pulmonary nodules. Surgical lung biopsy revealed cellular NSIP (nonspecific interstitial pneumonia) and LIP (lymphoid interstitial pneumonia). Case: A 30-year-old female presented to the hospital with progressive dyspnea for approximately 3 weeks that had worsened and was associated with decreased exercise tolerance. All routine laboratory workup was unremarkable. Inflammatory markers like CRP, ESR, LDH were slightly elevated. COVID-19 test was negative. A CT angiography of the thorax demonstrated an 8cm anterior right upper lobe pulmonary mass with numerous pulmonary nodules in bilateral lung fields. MRI of the brain, bone scan and CT scan of the abdomen and pelvis did not demonstrate any evidence of metastatic disease. A CT-guided core needle biopsy of the anterior lung mass was performed as well as several rheumatologic blood tests. Antinuclear antibody was positive with elevated RNP antibody 3.2 (reference <1), elevated autoantibodies to SS-A/Ro 6.5 (reference <1). The pathology of the core needle biopsy of the anterior lung mass was positive for thymoma. A video assisted thoracoscopic surgery of the lung was performed. The pathology of the pulmonary nodules was positive for overlapping features of cellular nonspecific interstitial pneumonia and LIP. The diagnoses of Sjogren's related interstitial lung disease and thymoma were made. She was treated with mycophenolate and prednisone. Discussion: NSIP is primarily idiopathic however it can be found in association with connective tissue disorders including Sjogren's disease, lupus, dermatomyositis, and polymyositis. CT findings for NSIP often show traction bronchiectasis with symmetric and bilateral ground-glass opacities with no honeycombing. This case above describes a patient with bilateral pulmonary nodules that were initially thought to be metastatic from her accompanying anterior chest wall mass. Although considered very rare, anterior chest wall masses that are associated with connective tissue diseases include thymoma, thymic cyst, teratoma, and thymic carcinoma. Our patient had a rare presentation of Sjogren associated interstitial lung disease and thymoma with atypical CT findings of NSIP.
{"title":"Interstitial Lung Disease Presenting with Bilateral Pulmonary Nodules","authors":"F. Mfone, O. Enilari, V. Pathak","doi":"10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2064","DOIUrl":"https://doi.org/10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2064","url":null,"abstract":"Introduction: Interstitial Lung Disease (ILD) is the heterogenous group of disease that share similar clinical, radiological and physiological findings and hence mostly grouped together. Radiologically, they present with varied findings which includes ground glass opacity, reticulations, honey combing, septal thickenings or the combination of these findings. Some specific combination could lead to the diagnosis of a particular ILD provided radiological findings match the clinical presentations. We present a patient who came with chronic cough and shortness of breath and CT chest showed anterior mediastinal mass with bilateral pulmonary nodules. Surgical lung biopsy revealed cellular NSIP (nonspecific interstitial pneumonia) and LIP (lymphoid interstitial pneumonia). Case: A 30-year-old female presented to the hospital with progressive dyspnea for approximately 3 weeks that had worsened and was associated with decreased exercise tolerance. All routine laboratory workup was unremarkable. Inflammatory markers like CRP, ESR, LDH were slightly elevated. COVID-19 test was negative. A CT angiography of the thorax demonstrated an 8cm anterior right upper lobe pulmonary mass with numerous pulmonary nodules in bilateral lung fields. MRI of the brain, bone scan and CT scan of the abdomen and pelvis did not demonstrate any evidence of metastatic disease. A CT-guided core needle biopsy of the anterior lung mass was performed as well as several rheumatologic blood tests. Antinuclear antibody was positive with elevated RNP antibody 3.2 (reference <1), elevated autoantibodies to SS-A/Ro 6.5 (reference <1). The pathology of the core needle biopsy of the anterior lung mass was positive for thymoma. A video assisted thoracoscopic surgery of the lung was performed. The pathology of the pulmonary nodules was positive for overlapping features of cellular nonspecific interstitial pneumonia and LIP. The diagnoses of Sjogren's related interstitial lung disease and thymoma were made. She was treated with mycophenolate and prednisone. Discussion: NSIP is primarily idiopathic however it can be found in association with connective tissue disorders including Sjogren's disease, lupus, dermatomyositis, and polymyositis. CT findings for NSIP often show traction bronchiectasis with symmetric and bilateral ground-glass opacities with no honeycombing. This case above describes a patient with bilateral pulmonary nodules that were initially thought to be metastatic from her accompanying anterior chest wall mass. Although considered very rare, anterior chest wall masses that are associated with connective tissue diseases include thymoma, thymic cyst, teratoma, and thymic carcinoma. Our patient had a rare presentation of Sjogren associated interstitial lung disease and thymoma with atypical CT findings of NSIP.","PeriodicalId":114116,"journal":{"name":"TP33. TP033 CASE REPORTS IN AUTOIMMUNE LUNG DISEASE","volume":"16 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":"127272704","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.a2036
A. Ross, A. Orfanakis
{"title":"The Kitchen Sink: Cardiac Arrest Requiring Veno-Venous Extracorporeal Membrane Oxygenation, Plasma Exchange, Steroids, and Rituximab in a Case of Granulomatosis with Polyangiitis","authors":"A. Ross, A. Orfanakis","doi":"10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2036","DOIUrl":"https://doi.org/10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2036","url":null,"abstract":"","PeriodicalId":114116,"journal":{"name":"TP33. TP033 CASE REPORTS IN AUTOIMMUNE LUNG DISEASE","volume":"33 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":"114241219","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.a2050
S. Vakharia, J. Carlson, S. Srivastava
{"title":"Moving to Breathe: Unmasked Rheumatoid Arthritis-Interstitial Lung Disease in a Patient Presenting with Heroin Overdose for Joint Pain","authors":"S. Vakharia, J. Carlson, S. Srivastava","doi":"10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2050","DOIUrl":"https://doi.org/10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2050","url":null,"abstract":"","PeriodicalId":114116,"journal":{"name":"TP33. TP033 CASE REPORTS IN AUTOIMMUNE LUNG DISEASE","volume":"122 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":"133889458","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.a2037
S. Nettboy, K. Wojnowski, J. Choi, M. Bhatia, P. Eftekhari
{"title":"Acute Pulmonary Renal Syndrome in a Critically Ill Lupus Patient and the Use of Plasma Exchange and Rituximab as Rescue Therapy","authors":"S. Nettboy, K. Wojnowski, J. Choi, M. Bhatia, P. Eftekhari","doi":"10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2037","DOIUrl":"https://doi.org/10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2037","url":null,"abstract":"","PeriodicalId":114116,"journal":{"name":"TP33. TP033 CASE REPORTS IN AUTOIMMUNE LUNG DISEASE","volume":"147 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":"114647841","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.a2061
G. Colbenson, C. Rose, F. Ernste, J. Ryu
{"title":"Successful Pregnancy Outcome with Connective Tissue Disease-Associated Interstitial Lung Disease","authors":"G. Colbenson, C. Rose, F. Ernste, J. Ryu","doi":"10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2061","DOIUrl":"https://doi.org/10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2061","url":null,"abstract":"","PeriodicalId":114116,"journal":{"name":"TP33. TP033 CASE REPORTS IN AUTOIMMUNE LUNG DISEASE","volume":"5 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":"121022847","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.a2038
K. Namasivayam, J. Stoll, K. Solanki, S. Hussain
{"title":"Diffuse Alveolar Hemorrhage and Active Nephropathy Secondary to Seronegative Lupus-Like Entity","authors":"K. Namasivayam, J. Stoll, K. Solanki, S. Hussain","doi":"10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2038","DOIUrl":"https://doi.org/10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2038","url":null,"abstract":"","PeriodicalId":114116,"journal":{"name":"TP33. TP033 CASE REPORTS IN AUTOIMMUNE LUNG DISEASE","volume":"368 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":"124620273","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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