CHEST Guidelines-A-26-Year-Old-Woman-With-Systemic-Lupus-Erythemato

A-26-Year-Old-Woman-With-Systemic-Lupus-Erythemato

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The document discusses a case study of a 26-year-old woman diagnosed with systemic lupus erythematosus (SLE) who presented with orthopnea and restrictive lung impairment. Initially diagnosed with SLE at 21, she was maintained on low doses of prednisone and mycophenolate. The patient experienced worsening symptoms including left shoulder pain and increasing orthopnea, requiring elevated sleeping positions. Diagnostic evaluations revealed a small left pleural effusion and severe restrictive lung function, specifically indicative of shrinking lung syndrome (SLS), characterized by reduced lung volumes and diaphragmatic dysfunction.<br /><br />The condition, although rare in SLE patients (0.5% prevalence), is primarily marked by progressive dyspnea, orthopnea, and pleuritic chest pain. The exact mechanisms of SLS are unclear, with potential contributory factors being diaphragmatic dysfunction, pleural inflammation, and possibly phrenic neuropathy. Treatments focus on aggressive immunosuppression using medications like prednisone, mycophenolate, and possibly rituximab. Supportive measures such as nocturnal noninvasive positive pressure ventilation (NIPPV) can help improve diaphragmatic function and respiratory outcomes by preventing nocturnal hypoventilation.<br /><br />The patient’s treatment with increased immunosuppression did not initially improve lung function. However, using NIPPV yielded significant symptomatic relief and improved pulmonary tests within a year. This case highlights the complexity of diagnosing and managing SLS in SLE and underscores the importance of early detection and multidisciplinary treatment strategies, including innovative uses of NIPPV to support diaphragmatic function along with conventional pharmacotherapy. Clinical insights emphasize thorough evaluation of diaphragm function in suspected cases and tailored treatment to prevent deterioration of respiratory function and quality of life.

Keywords

systemic lupus erythematosus

shrinking lung syndrome

orthopnea

restrictive lung impairment

diaphragmatic dysfunction

immunosuppression

noninvasive positive pressure ventilation

pleural effusion

phrenic neuropathy

multidisciplinary treatment