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Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
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Shrinking lung syndrome in systemic lupus erythematosus-scleroderma overlap
Vivek S Guleria1, Pradeep K Singh1, Puneet Saxena1
1Department of Internal Medicine, Armed Forces Medical College, Pune, Maharashtra, India.
Lung India : Official Organ of Indian Chest Society
|November 8, 2014
Summary
Shrinking lung syndrome (SLS), a rare systemic lupus erythematosus (SLE) complication, is presented in a patient with SLE-scleroderma overlap. This case highlights the importance of considering SLS in SLE patients with respiratory symptoms.
Area of Science:
- Rheumatology
- Pulmonology
- Internal Medicine
Background:
- Shrinking lung syndrome (SLS) is a rare manifestation of systemic lupus erythematosus (SLE), affecting approximately 0.5% of patients.
- The pathogenesis of SLS remains unclear, and treatment outcomes are variable, with only 77 cases documented.
- SLS in the context of SLE-scleroderma overlap has not been previously reported.
Purpose of the Study:
- To report the first documented case of Shrinking Lung Syndrome (SLS) in a patient with Systemic Lupus Erythematosus (SLE) and Scleroderma overlap.
- To emphasize the importance of recognizing SLS in SLE patients presenting with respiratory distress.
Main Methods:
- Case report of a patient with SLE-scleroderma overlap presenting with dyspnea, orthopnea, and pleuritic chest pain.
- Diagnostic evaluation included assessment for elevated hemidiaphragms and restrictive lung defects.
Main Results:
- The patient was diagnosed with Shrinking Lung Syndrome (SLS).
- Findings included elevated hemidiaphragms and a severe restrictive ventilatory defect.
Conclusions:
- Shrinking Lung Syndrome (SLS) is a rare but significant complication of Systemic Lupus Erythematosus (SLE).
- This case underscores the necessity for clinicians to consider SLS in SLE patients, particularly those with overlapping conditions like scleroderma, who exhibit respiratory symptoms.
- Early consideration of SLS can aid in timely diagnosis and management of respiratory compromise in this patient subset.
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