GLEICH SYNDROME K Habbal MD1, S Saunier BS2, G Akoghlanian MD1 1Department of Medicine, Louisiana State University Health Sciences Center, Lafayette, LA 2School of Medicine, LSU Health Sciences Center, New Orleans, LA
INTRODUCTION: Episodic angioedema with eosinophilia, also known as Gleich Syndrome is a rare disorder characterized by monthly intervals of spontaneously resolving urticaria, fever, angioedema, and dramatic eosinophilia. Gleich Syndrome generally responds very well to systemic corticosteroid treatment, and affected patients usually have a good clinical prognosis.
CASE: A 36-year-old male presented to clinic with intermittent joint pain and swelling, subjective fevers, and chronic non- productive cough for the past three years. Of note, he started a job as a construction site manager in West Texas and Arizona approximately 4 years prior to presentation. Initial labs revealed significant leukocytosis with eosinophilia, mildly elevated CRP, and normal sedimentation rate. Rheumatologic workup and imaging exhibited axillary lymphadenopathy, moderate splenomegaly, and bilateral alveolar consolidations of the mid lobes. Infectious disease evaluation revealed negative HIV and hepatitis screening, negative stool ova and parasites, and a positive coccidiomycosis IgM antibody although cultures and IgG were negative. Biopsy of an axillary lymph node exhibited reactive lymphoid hyperplasia with sinus histiocytosis and no signs of atypia or evidence of malignancy. After further evaluation, the patient admitted to experiencing frequent episodes of night sweats and occasional episodes of rash and generalized edema. After seven months of extensive infectious disease, hematology, oncology, allergy, and immunology workup, a diagnosis of Gleich Syndrome was finally achieved for this patient. He responded very well to corticosteroid therapy during disease flares. DISCUSSION: Episodic angioedema with eosinophilia (EAE) is a multilineage cycling disorder which is rare with less than 50 reported cases. Defined by periodic cycling eosinophilia and angioedema and preceded by rise in serum IL-5. Previously reported patients with definite EAE had a rise in IgM levels which is uncommon in Idiopathic Hypereosinophiilc Syndromes (HES). EAE is associated with the presence of aberrant and/or clonal T-cell populations and eosinophil-driven pathology. Aberrant clonal lymphocytes CD3−CD4+, were shown to produce increased type II cytokines driving eosinophilia. Previously studied subjects had increased CD3−CD4+ aberrant T cells at peak eosinophilia coinciding with symptoms. Although the intracellular cytokine profile in T-lymphocytes suggests that the eosinophil activation may be lymphocyte-driven, the etiology of the cycling in EAE is still unknown.
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