Abstract Introduction Granulomatous lymphocytic interstitial lung disease (GLILD) is a distinct clinicopathologic and radiologic entity most frequently observed in patients with common variable immunodeficiency (CVID). It is characterized by lymphoid proliferation and granulomatous inflammation within the lung parenchyma. Although classically associated with CVID, GLILD-like pathology has been rarely reported in autoimmune conditions such as Sjögren’s syndrome. We present a rare case of GLILD in a patient with Sjögren’s, illustrating the overlap between autoimmune-associated and immunodeficiency-related interstitial lung disease. Case Description A 67-year-old African-American woman with a history of lymphocytic interstitial pneumonia (LIP) secondary to Sjögren’s syndrome presented initially with progressive dyspnea over several months. Initial chest radiography was read as unremarkable yet non-contrast CT of the chest revealed bilateral cystic changes initially misinterpreted as emphysema. Autoimmune serologies confirmed SS-A and SS-B positivity. The patient had no smoking history or occupational exposures and significant bullous emphysema of unclear etiology. At her six-month follow-up, repeat CT imaging demonstrated new bilateral pulmonary nodules. She denied fever, night sweats, or weight loss. Pulmonary function testing revealed a moderate obstructive defect (FEV₁ 68%) with air trapping and reduced diffusing capacity (DLCO 46%). We proceeded with a robotic-assisted bronchoscopy with biopsies of the right middle and lower lobes, revealing chronic inflammation with necrotizing granulomas and multinucleated giant cells. Given the history of LIP and Sjögren’s, concern for lymphoproliferative disease prompted an elective video-assisted thoracoscopic (VATS) biopsy. Pathology demonstrated focal non-necrotizing granulomas with dense lymphoid infiltration, consistent with GLILD. The patient was started on systemic corticosteroids, resulting in symptomatic improvement. At three-month follow-up, pulmonary function testing showed a 15% increase in FEV₁ and DLCO. She was transitioned to azathioprine with a steroid taper and continues ongoing radiologic and clinical surveillance. Discussion This case represents a rare occurrence of GLILD in Sjögren’s syndrome. While GLILD is most commonly associated with CVID, only a limited number of cases have been described in autoimmune disease. Recognizing this uncommon overlap is critical for appropriate immunosuppressive management and to differentiate GLILD from infectious or malignant processes that may mimic it radiographically and histologically. This abstract is funded by: none
Pasiah et al. (Fri,) studied this question.