CRA98 A RARE CASE OF LUNG ADENOCARCINOMA IN PRIMARY SJOGREN SYNDROME WITH INTERSTITIAL LUNG DISEASE

Ahmad Adib Mohd Nasir, Noor Eliana Rozani, Nor Syamimi Mohd Isa, Haly Rozie Ahmad, Fatimah Azmah Mohammad, Leong Swee Wei, Mona Zaria Nasaruddin, Noorul Afidza Muhammad

Hospital Sultan Idris Shah, Serdang

Primary Sjogren Syndrome (pSS) is one of the common autoimmune disease worldwide affecting 0.5-1.0% of population. Interstitial Lung Disease associated with pSS (pSS-ILD) is well-known and it has been shown that pSS is also associated with malignancies especially non-Hodgkin B-cell lymphoma. Although the risk of lung malignancy increased in patients with ILD, lung adenocarcinoma in pSS is actually quite rare.

Here we present a case of lung adenocarcinoma associated with pss-ILD.

Mr S is a 67 year old Punjabi gentleman who was diagnosed with pSS back in 2023. He presented with complaints of dry eyes and mouth since 2014 and progressive worsening of dyspnoea since early 2023. On clinical examination he has velcro-like fine crepitations on aucultations of both lungs and a positive Schimmer Test of bilateral eyes. His Rheumatoid Factor (RF), Antinuclear Antibody (ANA), Extractable Nuclear Antigen (ENA) to SSA and SSB were all positive. Serial pulmonary function tests from 2023 until today remains stable but his 6-minute walk test showed deterioration with significant drop in oxygen saturations. Serial High Resolution Computed Tomography (HRCT) with multi disciplinary team discussion shows evidence of disease progression with fibrosis and lymph nodes enlargement. Due to high suspicion of lymphoma, he underwent an Endobronchial Ultrasound with Transbronchial Needle Aspiration (EBBUS-TBNA) and Cryobiopsy at the end of 2024. The histopathological examinations (HPE) suprisingly reveals metastatic adenocarcinoma with posistive thyroid transcription factor 1 (TTF-1) suggestive of lung primary. Next Generation Sequencing (NGS) shows a positive MET Exon 14  Skipping Mutation. He currently remains stable under multi-team managements.

This case highlighted that despite the most common malignancies associated with pSS-ILD is haematological malignancies i.e. lymphoma, few case reports has shown other malignancies can also be associated with pSS-ILD such as nasopharyngeal carcinoma (NPC). A recent study by Ying Li et. al. has shown that there is high probability of carcinogenesis induced by pulmonary inflammatory microenvironement in pSS that predisposed patients to lung adenocarcinoma. Management of such disease requires close multidisciplinary team expertises and monitoring.