Primary Sjögren’s Syndrome Associated Lymphoid Interstitial Pneumonia: The Enemy In Shadows

Sjögren's Syndrome and Pneumonia

Abstract

LIP is characterized by the interstitial infiltration of lung tissue, especially the interalveolar septum by polyclonal lymphocytes, plasma cells and histiocytes. This clinical entity is known for its association with primary Sjögren's syndrome (pSS). In this case presentation, we aim to provide an overview of the diagnosis and treatment processes with a clinical example of this rarely encountered condition. A 69-year-old female patient was referred to our rheumatology department with complaints of cough, shortness of breath, widespread joint pain and bilateral widespread cystic lesions and ground-glass opacities in her thoracic CT scan. The patient had no comorbidities other than a history of metastatic renal cell carcinoma, for which she underwent a left pneumonectomy five years ago. Serological tests revealed ANA > 1:320 and SS-A (Ro) positivity. Salivary gland biopsy showed lymphocytic sialadenitis. Due to the irregular, widespread peribronchial distribution of the cystic lesions seen on the thoracic CT, pSS-associated LIP was considered. Bronchoalveolar lavage (BAL) fluid was rich in lymphocytes but did not provide diagnostic information. In light of these results, the patient was started on treatment with methylprednisolone at a dose of 0.5 mg/kg/day and hydroxychloroquine. Azathioprine was added after tapering steroid and methylprednisolone stopped at 6 months. 4-9% of pSS patients are confronting LIP and 30% of underlying medical conditions in LIP is pSS. It should be noted that rare but potentially life-threatening lung involvements like LIP can develop in Sjögren's cases, and, as in our case, it may even be the initial presentation. Current results are indicating improvement with glucocorticoid+DMARD treatment, but randomized controlled studies are strongly needed.