HONEY, I SHRUNK THE LUNGS S Rashid MD, V. Nguyen MD Department of Internal Medicine, LSUHSC Shreveport, Louisiana
INTRODUCTION: Systemic Lupus Erythematosus (SLE) is an autoimmune inflammatory disease that can affect many organ systems including dermatologic, rheumatologic, renal, pulmonary, hematologic and central nervous system. The clinical course varies among patients and comprises of periods of relapses and remissions. We present a case of a patient with shrinking lung syndrome as a pulmonary complication secondary to lupus. CASE: Our patient is a 33-year old woman with history of stage V lupus nephritis diagnosed at age 19, hypertension, seizure disorder, smoking history who was admitted for a 7-month history of dyspnea on exertion and hypoxic respiratory failure. Physical exam was significant for decreased breath sounds bilaterally. Chest radiograph showed reduced lung volumes and prominent pulmonary artery. Chest computed tomography (CT) revealed linear atelectasis in bilateral lungs, however no evidence of interstitial lung disease. Pulmonary function test (PFT) revealed reduced lung volumes, increased FEV1/ FVC ratio, consistent with restrictive pattern of lung disease. Forced vital capacity was 29% of predicted, down from 34% 6-months prior. Sniff test was performed to evaluate for diaphragmatic paralysis was normal. DISCUSSION: This case highlights the importance of maintaining a high index of suspicion for various pulmonary manifestations of SLE. Some of the commonly presented complications include pleuritis, acute lupus pneumonitis, interstitial lung disease and pulmonary hypertension. Shrinking lung syndrome is a rare phenomenon defined by progressive dyspnea on exertion and pleuritic chest pain. The exact pathogenesis remains unclear; however, it is proposed that myositis affecting the diaphragms results in diaphragmatic elevation and decreased function. Diagnosis is based on progressive worsening in lung volumes and restrictive pattern on PFTs and lack of interstitial lung or pleural disease on chest CT. Treatment involves glucocorticoids and immunosuppressive therapy that may improve pulmonary symptoms and function. In our patient, PFT’s demonstrated a restrictive pattern and worsening in lung volumes over time without evidence of interstitial lung or pleural disease on chest CT, confirming shrinking lung syndrome. Our patient was started on corticosteroids for treatment.
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