A Case of Anti-Ro52 Antibody Myositis-Associated Interstitial Lung Disease
American Journal of Respiratory and Critical Care Medicine
; 205(1), 2022.
Article
in English
| EMBASE | ID: covidwho-1927718
ABSTRACT
Introduction:
Recognition of myositis-associated interstitial lung disease (ILD) has been increasing due to the recent identification of specific antibodies that are linked to a particular phenotype of myositis-ILD such as antisynthetase syndrome. The anti-Ro52 antibody has been found in multiple connective tissue diseases but its association with ILD is unclear. In fact, the literature on the phenotype of patients with an isolated anti-Ro52 antibody is very scarce. Case Presentation A 57-year-old retired firefighter with history of gastroesophageal reflux presented to the hospital with a 4-week history of cough and progressive dyspnea with no other symptoms. He had presented to an urgent care facility where he tested negative for SARs-CoV-2 and was given antibiotics and inhalers without a clinical response. Patient required 2L/min of oxygen on admission. He had no other signs of autoimmune disease or other organ involvement. Chest CT showed peripheral ground glass opacities with basilar reticulations and bronchiectasis (Figure 1). WBC and CPK were normal and aldolase was mildly elevated. Initial serologies revealed an ANA 1320 and a weakly positive p-ANCA with negative PR3/MPO antibodies. Patient was started on prednisone 60 mg and discharged five days later on mycophenolate mofetil (MMF) and 2L/min of oxygen. At 2-week follow-up, his extensive autoimmune panel showed a high titer of Anti-Ro52 antibody. An in depth ILD questionnaire revealed a family history of lupus and the remote use of a short course of steroids 20 years ago after a biceps biopsy showed polymyositis. On chart review, 10 years ago the patient had elevated CPK levels of 255 to 404, in the context of a nonspecific flank pain that resolved without intervention. At his 3-month follow-up, the patient still required 40mg of prednisone, reported dyspnea on exertion and required oxygen with ambulation. Rituximab was added to his regimen and his symptoms significantly improved;at 6-month follow-up, his FVC% improved from 53% to 70% and the patient was no longer on oxygen. Prednisone was titrated down to 10mg and he was continued on MMF 3g/day and Rituximab every 6 months.Discussion:
We present a patient with Anti-Ro52 antibody myositisassociated ILD who required 3 immunosuppressive agents to control his disease. This case adds to the very scarce literature on ILD secondary to Anti-Ro52 antibody and highlights the importance of an extensive antibody testing for patients with ILD of unclear etiology, for diagnostic and therapeutic purposes. Further studies describing the phenotype of these patients are warranted. (Figure Presented).
antibiotic agent; endogenous compound; fructose bisphosphate aldolase; immunosuppressive agent; mycophenolate mofetil; neutrophil cytoplasmic antibody; oxygen; prednisone; rituximab; adult; autoimmune disease; biceps brachii muscle; bronchiectasis; case report; clinical article; conference abstract; coughing; drug dose titration; drug therapy; dyspnea; exertional dyspnea; family history; fire fighter; flank pain; follow up; forced vital capacity; gastroesophageal reflux; ground glass opacity; human; inhaler; interstitial lung disease; male; medical record review; middle aged; mobilization; myositis; nonhuman; phenotype; polymyositis; questionnaire; serology; Severe acute respiratory syndrome coronavirus 2; systemic lupus erythematosus; thorax
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Databases of international organizations
Database:
EMBASE
Language:
English
Journal:
American Journal of Respiratory and Critical Care Medicine
Year:
2022
Document Type:
Article
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