Shrinking Lung Syndrome in a Young Female: A Rare Pulmonary Manifestation of Systemic Lupus Erythematosus

Abstract

Shrinking lung syndrome (SLS) is a rare pulmonary complication of autoimmune conditions. It has been sparsely described in the literature and its pathophysiology remains unclear. SLS is typically reported in patients with a history of systemic lupus erythematosus (SLE) who present with shortness of breath and chest pain associated with breathing. Chest imaging demonstrates no alveolar, interstitial, or pleural abnormalities. Pulmonary function tests (PFTs) are characterized by a restrictive pattern with reduced lung volumes. SLS is a diagnosis of exclusion and there are no validated criteria for the diagnosis. Evaluation requires extensive testing to rule out alternative causes of dyspnea and pleuritic chest pain. In this report, we present a case of SLS in a young African American woman.

Systemic Lupus Erythematosus-related Lung Disease

“…[63][64][65] Some patients can present with respiratory failure requiring mechanical ventilation. 66 Clinical examination is unremarkable in many patients, while other patients may present with rapid shallow breathing, limited chest wall expansion, use of the accessory muscles of respiration, and bibasilar crepitations due to basal atelectasis. 63,66 Although it predominantly impacts patients with SLE, it has been described in other autoimmune rheumatic diseases including primary Sjögren's syndrome, rheumatoid arthritis, polymyositis, and systemic sclerosis.…”

“…66 Clinical examination is unremarkable in many patients, while other patients may present with rapid shallow breathing, limited chest wall expansion, use of the accessory muscles of respiration, and bibasilar crepitations due to basal atelectasis. 63,66 Although it predominantly impacts patients with SLE, it has been described in other autoimmune rheumatic diseases including primary Sjögren's syndrome, rheumatoid arthritis, polymyositis, and systemic sclerosis. [67][68][69][70] SLS has a prevalence of 0.8 to 1.6%, though this is probably underreported due to underrecognition of the condition.…”

Combination treatment with rituximab and belimumab in shrinking lung syndrome in systemic lupus erythematosus

“…5 However, it is probable that various pathological mechanisms occur within the context of a dysfunctional immune response, given that medications that target the immune response have demonstrated clinical benefits. 6 Treatment usually involves oral corticosteroids and may be preceded by a short course of intravenous methylprednisolone, depending on initial severity, being effective in most cases. 5 For corticosteroid-refractory SLS, there is limited evidence on the optimal treatment approach.…”

“…5 For corticosteroid-refractory SLS, there is limited evidence on the optimal treatment approach. 5 While immunosuppressants, including cyclophosphamide, azathioprine, and methotrexate, have shown variable success in corticosteroid-refractory cases, 5,6 rituximab has emerged as a promising option in several case reports. 7,8 Our patient developed SLS despite previous ongoing treatment with corticosteroids and belimumab.…”