Abstract Introduction Anti-synthetase syndrome (ASS) is a subtype of idiopathic inflammatory myopathies, characterized by antibodies directed against aminoacyl- tRNA synthetases. Rheumatological diagnosis is often delayed due to the heterogeneous manifestations of connective tissue disorders (CTDs). Common myositis-specific antibodies in ASS include Anti-Jo-1 and non-Jo antibodies like anti-PL-7/PL-12. In most cases, ILD precedes the onset of myositis. Anti-PL-12 is mainly associated with ILD alone, absent or subclinical myositis, high morbidity and mortality due to progression to fibrotic ILD, higher rates of relapse, and treatment refractoriness. Description A 50-year-old female presented to a rheumatologist with over 1 year of xerostomia, dysphagia, chronic cough, decreased grip strength, pleuritic chest pain and dyspnea with persistent chest imaging infiltrates. She had been previously treated as a bacterial pneumonia. Physical examination revealed bibasilar lung crackles, periungual telangiectasias and steroid-responsive inflammatory dactylitis. Myositis antibody panel was positive for Anti-PL-12. Additionally, she had elevated erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) antinuclear antibody (ANA) and anti-Ro/SSA (traditionally present in Sjogren’s disease but associated with severe fibrotic ILD). The patient was promptly referred to pulmonology. Computed tomography revealed bibasilar peripheral reticulations with ground glass opacities and bronchiectasis, suggestive of cellular nonspecific interstitial pneumonia (NSIP) pattern with a component of organizing pneumonia (OP). Pulmonary function tests (PFTs) revealed a restrictive defect. She was started on steroids and Pneumocystis jirovecii (PJP) prophylaxis and was advised to continue follow up with her rheumatologist for a definite autoimmune diagnosis and possible initiation of steroid sparing immunosuppressants. Unfortunately, she has lost follow up and current clinical status is unknown. Discussion ASS is relatively uncommon, often underdiagnosed, especially when non-classic features are present. Radiological patterns of NSIP, OP, or a combination of both should trigger an investigation for autoimmune diseases including myositis-associated antibodies. Usual interstitial pneumonia (UIP) pattern is rarely present. Formal treatment guidelines are lacking, treatment should be individualized considering steroids in combination with steroid-sparing agents like azathioprine, mycophenolate mofetil, cyclosporine, cyclophosphamide, tacrolimus, immunoglobulins, rituximab, or plasma exchange in fulminant cases. Additional considerations include PJP prophylaxis, supplemental oxygen, vitamin D and calcium supplementation, and bisphosphonates. Education on pulmonary rehabilitation, smoking cessation, and preventive vaccinations can also improve patient outcomes. Longitudinal surveillance entails annual PFTs, monitoring for adverse effects of corticosteroids, evolution of myositis and other CTD features, opportunistic infections, malignancies, pulmonary hypertension, and progressive-ILD necessitating lung transplant. Despite the promising role of antifibrotics, more studies are needed to incorporate their use into practice. This abstract is funded by: None
Nehete et al. (Fri,) studied this question.