ISSN: 2578-4633
Authors: Lapitan LFP, Biong KE, Lapitan RL*, Tabora-Lacdao JML and Recto MST
Background: Clarkson’s disease, more known as idiopathic systemic capillary leak syndrome (SCLS) is a rare condition characterized by hypotensive episodes due to capillary hyperpermeability, which may be manifested by fluid accumulation in any organ or potential space, causing range of complications: respiratory failure (from pulmonary edema or pleural effusion), mesenteric hypoperfusion (from hepatic congestion and severe intestinal edema), compartment syndrome and rhabdomyolysis, and even shock from hypovolemia or cardiac tamponade. Very few cases of cardiac tamponade have been reported and certainly a serious and life-threatening complication. Case Summary: A 44-year-old female, reported to have intermittent episodes of angioedema aggravated by menstruation and infection, came in due to persistent hypotension. Patient presented with flu-like symptoms and tested positive for COVID – 19 infection. Initial laboratories revealed hemoconcentration, slightly elevated creatinine and hypoalbuminemia. Electrolytes and urinalysis were normal. 2D echocardiography showed large pericardial effusion with right atrial and ventricular collapse suggestive of cardiac tamponade, hence patient underwent emergency pericardial windowing. Initial chest X-ray was normal but repeat study showed pleural effusion on the 4th day of admission which was drained surgically. Additional diagnostic tests revealed negative for hepatitis, Mycoplasmal and Epstein-Barr virus. Direct and indirect Coomb’s test were also negative and Lupus panel was unremarkable. Serum free light chain panel showed suspicious for low level monotypic serum free lambda light chains (based only on the free light ratio). Immunopathology results showed no overt evidence of monoclonal bands. Protein electrophoresis pattern showed decreased albumin fraction and increased alpha, beta, and gamma fractions. C1 inhibitor was slightly elevated while serum tryptase was within normal values. Patient was managed as a case of idiopathic systemic capillary leak syndrome or Clarkson’s disease. Aside from judicious fluid resuscitation and vasopressors assist Methylprednisolone pulse therapy (MPPT) and intravenous immunoglobulin (IVIg) were administered. Patient was discharged with tapering doses of oral steroids and scheduled monthly IV immunoglobulin infusion. Conclusion: Cardiac tamponade, an uncommon yet severe and life-threatening complication of systemic capillary leak syndrome (SCLS) or Clarkson’s disease, requires immediate intervention. While the exact cause remains unclear, research suggests that during acute SCLS episodes and hyperinflammatory states, fluid accumulation in the pericardial space can impede ventricular filling, leading to hemodynamic compromise.
Keywords: Cardiac Tamponade; Hemoconcentration; Hypoalbuminemia; COVID-19; Creatinine; Echocardiography; Chest Xray
