The American journal of the medical sciences
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Review Case Reports
Severe eosinophilic granulomatosis with polyangiitis responding to a combination of rituximab and mepolizumab.
Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss Syndrome, is a multisystem antineutrophil cytoplasmic antibody (ANCA) positive vasculitis, characterized by the presence of chronic rhinosinusitis, asthma and prominent peripheral blood eosinophilia. Although the most commonly involved organ is the lung, followed by the skin, EGPA can affect any organ system. Herein, we present the complicated case of an 18-year-old male patient with severe life-threatening EGPA, with central nervous system, cardiac and gasterointestinal involvement, which was resistant to initial treatment with glucocorticoids and cyclophosphamide. The patient responded well, achieving complete remission after the addition of rituximab and mepolizumab to glucocorticoids and cyclophosphamide.
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Review Case Reports
Bilateral adrenal hemorrhage: A rare presentation of catastrophic anti-phospholipid syndrome.
Catastrophic anti-phospholipid syndrome (CAPS) is characterized by microvascular thrombosis in multiple sites leading to multi-organ damage. It is a rare and fatal complication of antiphospholipid syndrome (APS). We present a rare case of CAPS that presented with bilateral (b/l) adrenal hemorrhage making the diagnosis challenging in this otherwise rare disease. ⋯ Patient had a fulminant disease course in which she had thrombotic manifestations involving multiple organ systems. This case was especially challenging as the patient's bilateral adrenal hemorrhage was the first manifestation of CAPS; the diagnosis of APS had to be made while treatment for presumed CAPS was emergently commenced for this life-threatening disease. Key to managing this condition is having a high index of suspicion for the diagnosis in patients presenting with multi-organ failure and multiple thromboses and hemorrhage.