A Rare Case of Steroid-dependent Rheumatoid Lung Disease Without Joint Manifestations

Document Type

Conference Proceeding

Publication Date

5-21-2024

Publication Title

Am J Respir Crit Care Med

Abstract

Introduction:Rheumatoid arthritis (RA) is a systemic inflammatory disorder with the most common manifestation extra-articular manifestation being lung involvement. RA can affect any lung compartment with varying presentation. RA-Interstitial lung disease (RA-ILD) is associated with significant morbidity and mortality. Lung involvement in RA typically occurs following articular manifestation, although it may occur prior to joint manifestation. Corticosteroids and Disease modifying anti-rheumatic drugs (DMARDS), have been remarked as mainstay of treatment. Case Presentation:60-year-old Female with significant past medical history of COVID-19 pneumonia 1 year prior, presented to the hospital due to progressively worsening exertional dyspnea of 3 years duration. On presentation, patient was afebrile, hypoxic to 88% requiring 2L nasal cannula, and normotensive. Physical exam was remarkable for female in no acute distress, and inspiratory crackles in bilateral lung fields. On further questioning she reported dry cough but denied fevers, chills, body aches, and chest pain. She denied sick contacts, tobacco, or vaping use. She reported significant social history of working on poultry farm but denied other environmental exposures. CTA chest was ordered in emergency department and showed bilateral ground glass opacities without pulmonary embolism. A high-resolution CT chest was subsequently ordered which showed increased interstitial marking in periphery of both lungs suggestive of chronic interstitial lung disease. Patient was started on high dose corticosteroids and admitted to medicine service with pulmonary consultation. Extensive work-up including infectious, hypersensitivity pneumonitis and auto-immune panel were ordered but resulted as negative with exception of isolated elevation in rheumatoid factor, Anti-CCP antibodies and inflammatory markers. Patient was ultimately diagnosed with Rheumatoid Arthritis associated lung disease (RA-ILD) with plan for bronchoscopy and biopsy outpatient pending clinical improvement. She was discharged on high-dose corticosteroid with taper but had repeated exacerbation of underlying pulmonary symptoms with any attempts to taper corticosteroids precluding bronchoscopy. Follow-up imaging showed continuous progression of diffuse interstitial fibrosis with non-specific interstitial pneumonia (NSIP) pattern. Patient was ultimately started on Mycophenolate in addition to corticosteroids due to disease progression on high dose corticosteroid therapy, with improvement in pulmonary symptoms. Discussion: Treatment strategies in RA-ILD have not been well-studied. Initiation or augmentation of treatment depends on initial disease severity and disease progression. Corticosteroids and other immunosuppressive medications remain the mainstay of treatment, although studies are lacking. This case demonstrates that subset of patients with RA-ILD, particularly without join manifestations may be poorly responsive to corticosteroids and may benefit from early initiation of concomitant DMARD therapy.

Volume

209

Issue

9

First Page

A1979

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