Abstract Introduction Lymphocytic interstitial pneumonia (LIP) is a rare form of interstitial lung disease characterized by diffuse lymphocytic interstitial infiltration often associated with systemic autoimmune disease and infrequently associated with pulmonary hypertension. We present a case of newly diagnosed connective tissue disease linked to symptoms of pulmonary arterial hypertension (PAH) with characteristic radiographic findings of LIP. Case Presentation A 42-year-old woman with tobacco use presented with dyspnea, cold sensitivity in her finger tips, and dry mouth. CT angiography of the chest revealed widespread variable pneumatocysts with an enlarged PA trunk diameter and RV/LV ratio 1. Echocardiogram confirmed RV dilation with estimated PA systolic pressure 32 mmHg and normal LV function. Labs were pertinent for SS-A 76, RF 15, ANA 1:320 speckled pattern. An outpatient V/Q scan was unremarkable and a right heart cath confirmed pre-capillary pulmonary hypertension: RA 11, RV 80/8, PA 84/40, PCWP 15 mmHg, TDCO 5.9/CI 2.9, Fick CO 4.6/2.3, PVR 6.27 WU. She was started on torsemide, tadalafil, and macitentan with referral to rheumatology. Discussion LIP is a cystic lung disease caused by an inflammatory reaction and occurs more often in association with autoimmune conditions compared to infectious causes (2). The inflammatory process results in interstitial infiltration of polyclonal B and T cells and CT manifestations include ground glass attenuation, subpleural micronodules, and interlobular septal thickening which was seen in our patient (4). Our patient also demonstrated evidence of pulmonary hypertension which was likely the primary cause of her dyspnea which would be categorized as WHO group 1 PAH associated with underlying connective tissue disease. Notably, PAH has a reported incidence rate of less than 2% in both Sjogrens and SLE cases. Therefore, a high index of suspicion is necessary to diagnose pulmonary hypertension in the setting of radiographic evidence of LIP associated with connective tissue disease. Conclusion Connective tissue diseases such as Sjogren’s and Lupus can present with isolated pulmonary manifestations including both parenchymal and vascular changes, manifesting as LIP and group 1 PAH. Early recognition, diagnosis, and concomitant treatment with continued pulmonary surveillance can slow progression of disease leading to improved patient outcomes. This abstract is funded by: none
Phan et al. (Fri,) studied this question.