Abstract Introduction Interstitial lung disease (ILD) is a severe manifestation of connective tissue diseases (CTD) and rarely occurs in Systemic Lupus Erythematosus (SLE). Symptomatic ILD as an initial manifestation of SLE is even rarer, which presents a therapeutic challenge due to limited data and no established recommendations. Case Description A 41-year old female presented with 1 year history of dry cough, progressive exertional dyspnea and pleuritic chest pain. Workup for pulmonary tuberculosis (PTB) was negative. She was started on LABA/ICS and antihistamines for presumed asthma and allergic rhinitis with minimal relief. Chest CT scan revealed subpleural reticular and ground glass densities with cystic bronchiectasis and honeycombing predominantly in both lower lobes, consistent with ILD, UIP pattern. 6 Minute Walk Test was only at 288 meters with Borg scale 2 at the last minute. Pulmonary function test showed normal spirometry - FEV1 of 1.8L, FVC of 2.05L, FEV1/FVC ratio 87%, Total lung capacity (TLC) 3.5L 80%, but diminished DLco at 51%. She was referred to an ILD specialist, who advised antifibrotic therapy for IPF that was not started due to financial constraints and was concurrently managed as PTB Clinically Diagnosed. Serial chest CT scan findings however did not improve. Transthoracic echocardiography with Doppler was unremarkable. Seven months into the course she developed joint pains, morning stiffness and dysphagia. Physical examination revealed tenderness of bilateral metacarpophalangeal, proximal and distal interphalangeal joints. Rheumatology referral was done with findings consistent with SLE with Nephritis: positive ANA IF 1:5120 with nuclear fine specked pattern; anti dsDNA 27.69 (borderline positive); negative for Anti-CCP, Anti-Scl 70, Anti-RNP, Anti-Jo-1; positive proteinuria and microscopic hematuria on urinalysis. She was started on induction therapy with hydroxychloroquine, mycophenolate mofetil and high-dose corticosteroids. Meanwhile, she completed her pulmonary rehabilitation sessions. Serial Chest CT after 3 months remained stable, with improvement in 6MWT to 318m, Borg scale 1 and PFT parameters: FEV1 2.17 L, FVC 2.5L, TLC 3.53L 81%, DLCO 54%. She is due for surveillance chest CT and PFT monitoring post induction phase. Discussion ILD as initial feature of SLE is rare and portends poor prognosis. This case highlights that SLE should be considered in patients presenting with ILD, regardless of systemic involvement. To our knowledge, this is the first documented case of SLE- associated ILD, and with ILD as its initial manifestation in the Philippines. This abstract is funded by: None
Teves et al. (Fri,) studied this question.