Shrinking lung syndrome: a rare manifestation of systemic lupus erythematosus

SESSION TITLE: Autoimmune Diffuse Lung Disease Case PostersSESSION TYPE: Case Report PostersPRESENTED ON: 10/19/2022 12:45 pm - 01:45 pmINTRODUCTION: Shrinking lung syndrome (SLS) is an infrequently reported manifestation of systemic lupus erythematosus (SLE) with 0.5-1% prevalence among the lupus population. It usually presents with dyspnea of unexplained origin, hemidiaphragm elevation on imaging, and is a diagnosis of exclusion.CASE PRESENTATION: A 63-year-old-male, with SLE, diagnosed 15 years ago, currently on hydroxychloroquine and prednisone, presented with dyspnea for 1 year and intermittent sharp chest pain with deep inspiration. The patient was a non-smoker. He was treated for asthma with minimal improvement then referred to our center for persistence of symptoms. Physical exam was significant for reduced breath sounds at bilateral bases. Basic laboratory workup was normal. CT chest showed bilateral chronic minimal pleural thickening, scattered chronic parenchymal calcified granulomas, and elevated right hemidiaphragm. These imaging findings did not correlate with his degree of dyspnea. Chest fluoroscopy did not show diaphragmatic paralysis but demonstrated right hemidiaphragm elevation 1½ rib interspaces above left. Pulmonary function testing (PFT) was consistent with severe restrictive lung disease (FEV1 47%, FVC 48%). Diffusion capacity was also reduced to 56%. Maximum inspiratory (37%) and expiratory (36%) pressures were also decreased. The diagnosis of SLS was made and the patient was started on a higher dose of steroids.DISCUSSION: Pleuropulmonary involvement in SLE affects about 80% of patients, most commonly with pleuritis, pulmonary hypertension, or chronic interstitial lung disease. SLS is a rare occurrence with just 0.5 -1% prevalence in lupus patients. SLS is characterized by unexplained dyspnea, decreased lung volumes, restrictive lung physiology, and elevation of hemidiaphragm in absence of interstitial, alveolar, or vascular pulmonary disease. The challenging part is it is a diagnosis of exclusion, which requires in-depth testing. Patients usually present with dyspnea and pleuritic chest pain. Common imaging findings are basal atelectasis and elevated hemidiaphragm. PFT will show a restrictive pattern with a median decrease of 50% of lung volume. Pathogenesis is not completely clear, but diaphragmatic dysfunction either due to nerve involvement or myopathy is postulated. Treatment is targeted towards underlying autoimmune diseases commonly with steroids. Immunosuppressive agents like rituximab, beta-agonists, and theophylline are reserved for non-responsiveness to steroids. Monoclonal antibodies like Belimumab have been tried where side effects are a limiting factor, but there is limited literature in terms of efficacy in SLS.CONCLUSIONS: Increased awareness and suspicion about SLS in the subset of patients with SLE presenting with dyspnea is important for early diagnosis, especially in the outpatient setting.Reference #1: Calderaro, D. C., & Ferreira, G. A. (2012). Presentation and prognosis of shrinking lung syndrome in systemic lupus erythematosus: report of four cases. Rheumatology International, 32(5), 1391–1396.Reference #2: Choudhury, S., Ramos, M., Anjum, H., Ali, M., & Surani, S. (2020). Shrinking Lung Syndrome: A Rare Manifestation of Systemic Lupus Erythematosus. Cureus, 12(5), e8216.Reference #3: Guleria, V. S., Singh, P. K., Saxena, P., & Subramanian, S. (2014). Shrinking lung syndrome in systemic lupus erythematosus-scleroderma overlap. Lung India: Official Organ of Indian Chest Society, 31(4), 407–409.DISCLOSURES: No relevant relationships by Awais BajwaNo relevant relationships by Himanshu BhardwajNo relevant relationships by SYED HUSSAINNo relevant relationships by Miloni Parmar SESSION TITLE: Autoimmune Diffuse Lung Disease Case Posters SESSION TYPE: Case Report Posters PRESENTED ON: 10/19/2022 12:45 pm - 01:45 pm INTRODUCTION: Shrinking lung syndrome (SLS) is an infrequently reported manifestation of systemic lupus erythematosus (SLE) with 0.5-1% prevalence among the lupus population. It usually presents with dyspnea of unexplained origin, hemidiaphragm elevation on imaging, and is a diagnosis of exclusion. CASE PRESENTATION: A 63-year-old-male, with SLE, diagnosed 15 years ago, currently on hydroxychloroquine and prednisone, presented with dyspnea for 1 year and intermittent sharp chest pain with deep inspiration. The patient was a non-smoker. He was treated for asthma with minimal improvement then referred to our center for persistence of symptoms. Physical exam was significant for reduced breath sounds at bilateral bases. Basic laboratory workup was normal. CT chest showed bilateral chronic minimal pleural thickening, scattered chronic parenchymal calcified granulomas, and elevated right hemidiaphragm. These imaging findings did not correlate with his degree of dyspnea. Chest fluoroscopy did not show diaphragmatic paralysis but demonstrated right hemidiaphragm elevation 1½ rib interspaces above left. Pulmonary function testing (PFT) was consistent with severe restrictive lung disease (FEV1 47%, FVC 48%). Diffusion capacity was also reduced to 56%. Maximum inspiratory (37%) and expiratory (36%) pressures were also decreased. The diagnosis of SLS was made and the patient was started on a higher dose of steroids. DISCUSSION: Pleuropulmonary involvement in SLE affects about 80% of patients, most commonly with pleuritis, pulmonary hypertension, or chronic interstitial lung disease. SLS is a rare occurrence with just 0.5 -1% prevalence in lupus patients. SLS is characterized by unexplained dyspnea, decreased lung volumes, restrictive lung physiology, and elevation of hemidiaphragm in absence of interstitial, alveolar, or vascular pulmonary disease. The challenging part is it is a diagnosis of exclusion, which requires in-depth testing. Patients usually present with dyspnea and pleuritic chest pain. Common imaging findings are basal atelectasis and elevated hemidiaphragm. PFT will show a restrictive pattern with a median decrease of 50% of lung volume. Pathogenesis is not completely clear, but diaphragmatic dysfunction either due to nerve involvement or myopathy is postulated. Treatment is targeted towards underlying autoimmune diseases commonly with steroids. Immunosuppressive agents like rituximab, beta-agonists, and theophylline are reserved for non-responsiveness to steroids. Monoclonal antibodies like Belimumab have been tried where side effects are a limiting factor, but there is limited literature in terms of efficacy in SLS. CONCLUSIONS: Increased awareness and suspicion about SLS in the subset of patients with SLE presenting with dyspnea is important for early diagnosis, especially in the outpatient setting. Reference #1: Calderaro, D. C., & Ferreira, G. A. (2012). Presentation and prognosis of shrinking lung syndrome in systemic lupus erythematosus: report of four cases. Rheumatology International, 32(5), 1391–1396. Reference #2: Choudhury, S., Ramos, M., Anjum, H., Ali, M., & Surani, S. (2020). Shrinking Lung Syndrome: A Rare Manifestation of Systemic Lupus Erythematosus. Cureus, 12(5), e8216. Reference #3: Guleria, V. S., Singh, P. K., Saxena, P., & Subramanian, S. (2014). Shrinking lung syndrome in systemic lupus erythematosus-scleroderma overlap. Lung India: Official Organ of Indian Chest Society, 31(4), 407–409. DISCLOSURES: No relevant relationships by Awais Bajwa No relevant relationships by Himanshu Bhardwaj No relevant relationships by SYED HUSSAIN No relevant relationships by Miloni Parmar