Allergy Asthma and Clinical Immunology· 2026Q2· Case report
Chronic systemic capillary leak syndrome with monoclonal kappa light chain gammopathy responsive to intravenous immunoglobulin: a case report
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- Q2SCImago
- 2026year
Short summary
A 39-year-old woman with a 15-year history of chronic, episodic limb edema and sweating, previously unresponsive to standard treatments, achieved complete symptom resolution with monthly high-dose intravenous immunoglobulin (IVIG) therapy for monoclonal kappa light chain gammopathy.
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Key points
- A 39-year-old woman presented with a 15-year history of chronic, episodic limb edema and sweating, lacking classic SCLS symptoms like hypotension.
- The patient was diagnosed with monoclonal kappa light chain gammopathy.
- Monthly high-dose intravenous immunoglobulin (IVIG) therapy resulted in complete resolution of swelling and marked reduction in sweating after five months.
- This case suggests IVIG may be a viable treatment option for chronic or atypical presentations of monoclonal gammopathy-associated SCLS.
AI-generated from the title and abstract; the full text is not read.
Abstract
Abstract Background Systemic capillary leak syndrome (SCLS) is a rare disorder characterized by transient plasma extravasation due to endothelial barrier dysfunction. It may occur idiopathically (ISCLS) or in association with monoclonal gammopathy (MG-CLS), a form also known as Clarkson’s disease. Case presentation We describe a 39-year-old woman with a 15-year history of episodic, symmetric limb edema and profuse sweating, unresponsive to antihistamines or corticosteroids over several years. Episodes were chronic and lacked hypotension or pruritus. Laboratory workup revealed monoclonal kappa light chain gammopathy and borderline hemoconcentration. Monthly high-dose intravenous immunoglobulin (IVIG, 2 g/kg) was initiated. Improvement was noted by the fourth infusion, with complete resolution of swelling and marked reduction in sweating after five months of therapy. IVIG was well tolerated apart from mild infusion-related headache. Conclusions This case illustrates that MG-CLS may present as a chronic disorder without the classic triad and may respond favorably to IVIG. In selected patients with mild or atypical presentations, a therapeutic trial of IVIG may be considered.
The authors' abstract, as published at the source. Allergy Asthma and Clinical Immunology, 2026 · DOI ↗
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ImmunologyImmunology and Microbiology