Abstract Introduction Sjogren’s syndrome (SS) is a systemic autoimmune disease with several potential pulmonary manifestations including but not limited to interstitial lung diseases (ILDs), non-Hodgkin lymphomas, and, rarely, thromboembolic disease or pulmonary hypertension. While approximately 10% to 20% of patients with SS experience clinically significant lung disease, a far rarer complication is the development of pulmonary amyloidosis whose radiological findings of nodules and cysts can mimic malignancy. We present the case of a 66-year-old woman with SS whose evaluation for progressive dyspnea led to the diagnosis of localized pulmonary amyloidosis and associated significant pulmonary hypertension (PH). Case Description A 66-year-old woman with a recent diagnosis of SS was hospitalized for worsening dyspnea. She had already been started on hydroxychloroquine by her rheumatologist prior to the admission.A chest CT revealed numerous bilateral nodules, cystic spaces, ground-glass opacities, and lymphadenopathy. These findings were atypical for classic lymphocytic interstitial pneumonia (LIP) and raised high suspicion for malignancy or a diffuse infiltrative disease. Pulmonary function testing showed a moderate restrictive defect with a severely reduced diffusing capacity (DLCO 31%). A navigational bronchoscopy with biopsy was performed, confirming amyloidosis via positive Congo red staining and, critically, ruling out MALT lymphoma. A subsequent hematology evaluation found no evidence of systemic amyloidosis. A transthoracic echocardiogram was notable for preserved left ventricular function and showed no structural changes suggestive of cardiac amyloid involvement, but it did reveal estimated pulmonary artery systolic pressure of 68 mmHg. Given the severely low DLCO and exclusion of systemic disease, a multidisciplinary discussion led to the initiation of prednisone therapy to treat the underlying inflammatory, autoimmune-driven process. The patient subsequently reported symptomatic improvement. Discussion This case highlights the diagnostic challenge of SS-associated pulmonary amyloidosis. The patient’s imaging, with its predominance of dense nodules and masses rather than the GGOs, typical of LIP, pointed toward a differential of malignancy or an infiltrative process like amyloidosis. The diagnostic process focused on two critical exclusions: systemic amyloidosis and, given the extensive lymphadenopathy, MALT lymphoma. Ruling out both was essential, given the high, persistent lymphoma risk in SS. The hematology workup and reassuring echocardiogram confirmed the amyloidosis was a localized process. This allowed the multidisciplinary team to target the nodular amyloidosis itself as the active, inflammatory Sjogren’s manifestation with prednisone. This case demonstrates the necessity of both a multidisciplinary approach for management and vigilant long-term surveillance for lymphoma, a risk that persists and may be farther heightened by future immunomodulatory therapy. This abstract is funded by: None
Tun et al. (Fri,) studied this question.