Abstract Background: Systemic Capillary Leak Syndrome (SCLS) is a rare and life-threatening condition characterized by reversible plasma extravasation, hypotension, hemoconcentration, and hypoalbuminemia. Clinical Description: An 8-year-old girl presented with prolonged fever, vomiting, abdominal pain associated with progressive muscle weakness. Work-up before presentation to us had shown transaminitis and evidence of appendicitis with peritonitis on computed tomography. However, the child was referred due to associated unexplainable features such as generalized edema, limb weakness, hypertension and thrombocytopenia. Examination showed fever, pallor, anasarca, tachycardia with low volume pulses, reduced power and tendon reflexes, with generalized muscle tenderness, and a lacy cutaneous rash over upper chest. Management and Outcome: Investigations revealed neutrophilic leukocytosis, hypoalbuminemia, elevated liver enzymes, creatinine phosphokinase (19,690IU/L) and lactate dehydrogenase. Magnetic Resonance Imaging of thighs demonstrated diffuse myositis. Serology was positive for ANA and anti-Jo-1 antibodies, suggesting JDM with possible overlap syndrome. Despite methylprednisolone and fluid resuscitation, the child went into shock, acute kidney injury and fluid overload, necessitating mechanical ventilation and kidney support therapy. Following transient improvement with high-dose immunosuppression and intravenous immunoglobulin, she developed Acinetobacter pneumonia and remained ventilator dependent. She further developed disseminated aspergillosis, requiring intravenous voriconazole. Her prolonged PICU stay was complicated by critical illness myopathy and was transferred to another low-cost centre, but succumbed to pulmonary hemorrhage soon after. Conclusion: This case highlights the complexity of managing autoimmune disease complicated by SCLS. Early recognition, immunomodulation, and aggressive supportive care are crucial. However, prolonged immunosuppression increases the risk of opportunistic infections, often with fatal outcomes.
Mandal et al. (Wed,) studied this question.