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Interstitial Lung Disease Secondary to Antisynthetase Syndrome Presenting as Acute Respiratory Failure
October 2015, Vol 148, No. 4_MeetingAbstracts
Diffuse Lung Disease | October 2015
Interstitial Lung Disease Secondary to Antisynthetase Syndrome Presenting as Acute Respiratory Failur Monia Werlang, MD; Isabel Mira-Avendano, MD Mayo Clinic, Jacksonville, FL Chest. 2015;148(4_MeetingAbstracts):404A. doi:10.1378/chest.2281566
Abstract SESSION TITLE: Diffuse Lung Disease Student/Resident Case Report Posters SESSION TYPE: Student/Resident Case Report Poster PRESENTED ON: Tuesday, October 27, 2015 at 01:30 PM - 02:30 PM INTRODUCTION: Antisynthetase Syndrome (ASS) is a recently recognized disorder characterized by an inflammatory myositis associated with interstitial lung disease and antisynthetase antibodies. Diagnosis of ASS is challenging due to its rarity and lack of uniform published data, leading to unclearly defined diagnostic criteria. We will discuss an unusual case of ASS who presented with acute hypoxemic respiratory failure. CASE PRESENTATION: A seventy-three-year-old non-smoking male was admitted to the hospital with nasal congestion, cough and general malaise. He had no history of lung disease or unusual exposures. He was diagnosed with community acquired pneumonia and treated with antibiotics without improvement. He later developed acute respiratory failure, requiring orotracheal intubation. Chest computed tomography (CT) showed areas of ground-glass opacities mixed with areas of consolidation in the lower lobes, mostly on the right side. (PICTURE1) He was treated with multiple antibiotics and high doses of methylprednisone. Patient had prolonged hospitalization and slow recovery, ultimately being discharged on no medications and with unclear etiology of his symptoms. Three weeks later he was readmitted with a new episode of acute respiratory failure and was given corticosteroids with significant improvement in his symptoms. Chest CT was performed after this hospitalization and showed persistent areas of ground glass opacities over the right lung, with areas suggestive of fibrotic changes and possible traction bronchiectasis. Further workup revealed positive SSA-Ro and Scl-70 antibodies. Additional testing showed strong positivity for anti-threonyl tRNA synthetase (anti-PL7) antibody. Repeated pulmonary function tests (PFT) were performed with significant improvement while on high dose steroid treatment and worsening when tapering was attempted. Patient was ultimately diagnosed with antisynthetase syndrome and was started on mycophenolate as a steroid-sparing agent. His clinical status, chest CT findings (PICTURE2) and PFT results improved. DISCUSSION: The severity and prognosis in patients with anti-PL-7 ASS is very heterogenous. Our patient illustrates a case where isolated interstitial lung disease without other rheumatologic manifestations responded very well to immunosuppressive therapy.
CONCLUSIONS: A lack of screening for anti-PL7 antibodies is a probable reason for underestimations of anti-PL7 positivity. Raising awareness about ASS and learning how to make the diagnosis in initital stages can have a huge impact, as the prognosis can be much improved with early treatment. Reference #1: Yamasaki, Y., et al., Unusually high frequency of autoantibodies to PL-7 associated with milder muscle disease in Japanese patients with polymyositis/dermatomyositis.Arthritis and rheumatism, 2006. 54(6): p. 2004-9. Reference #2: Hervier, B., et al., Antisynthetase syndrome positive for anti-threonyl-tRNA synthetase (anti-PL7) antibodies. The European respiratory journal, 2011. 37(3): p. 714-7 DISCLOSURE: The following authors have nothing to disclose: Monia Werlang, Isabel Mira-Avendano
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Diffuse Lung Disease | October 2015
Interstitial Lung Disease Secondary to Antisynthetase Syndrome Presenting as Acute Respiratory Failur Monia Werlang, MD; Isabel Mira-Avendano, MD Mayo Clinic, Jacksonville, FL Chest. 2015;148(4_MeetingAbstracts):404A. doi:10.1378/chest.2281566
Abstract SESSION TITLE: Diffuse Lung Disease Student/Resident Case Report Posters SESSION TYPE: Student/Resident Case Report Poster PRESENTED ON: Tuesday, October 27, 2015 at 01:30 PM - 02:30 PM INTRODUCTION: Antisynthetase Syndrome (ASS) is a recently recognized disorder characterized by an inflammatory myositis associated with interstitial lung disease and antisynthetase antibodies. Diagnosis of ASS is challenging due to its rarity and lack of uniform published data, leading to unclearly defined diagnostic criteria. We will discuss an unusual case of ASS who presented with acute hypoxemic respiratory failure. CASE PRESENTATION: A seventy-three-year-old non-smoking male was admitted to the hospital with nasal congestion, cough and general malaise. He had no history of lung disease or unusual exposures. He was diagnosed with community acquired pneumonia and treated with antibiotics without improvement. He later developed acute respiratory failure, requiring orotracheal intubation. Chest computed tomography (CT) showed areas of ground-glass opacities mixed with areas of consolidation in the lower lobes, mostly on the right side. (PICTURE1) He was treated with multiple antibiotics and high doses of methylprednisone. Patient had prolonged hospitalization and slow recovery, ultimately being discharged on no medications and with unclear etiology of his symptoms. Three weeks later he was readmitted with a new episode of acute respiratory failure and was given corticosteroids with significant improvement in his symptoms. Chest CT was performed after this hospitalization and showed persistent areas of ground glass opacities over the right lung, with areas suggestive of fibrotic changes and possible traction bronchiectasis. Further workup revealed positive SSA-Ro and Scl-70 antibodies. Additional testing showed strong positivity for anti-threonyl tRNA synthetase (anti-PL7) antibody. Repeated pulmonary function tests (PFT) were performed with significant improvement while on high dose steroid treatment and worsening when tapering was attempted. Patient was ultimately diagnosed with antisynthetase syndrome and was started on mycophenolate as a steroid-sparing agent. His clinical status, chest CT findings (PICTURE2) and PFT results improved. DISCUSSION: The severity and prognosis in patients with anti-PL-7 ASS is very heterogenous. Our patient illustrates a case where isolated interstitial lung disease without other rheumatologic manifestations responded very well to immunosuppressive therapy.
CONCLUSIONS: A lack of screening for anti-PL7 antibodies is a probable reason for underestimations of anti-PL7 positivity. Raising awareness about ASS and learning how to make the diagnosis in initital stages can have a huge impact, as the prognosis can be much improved with early treatment. Reference #1: Yamasaki, Y., et al., Unusually high frequency of autoantibodies to PL-7 associated with milder muscle disease in Japanese patients with polymyositis/dermatomyositis.Arthritis and rheumatism, 2006. 54(6): p. 2004-9. Reference #2: Hervier, B., et al., Antisynthetase syndrome positive for anti-threonyl-tRNA synthetase (anti-PL7) antibodies. The European respiratory journal, 2011. 37(3): p. 714-7 DISCLOSURE: The following authors have nothing to disclose: Monia Werlang, Isabel Mira-Avendano
No Product/Research Disclosure Information