Hypogammaglobulinemic sprue is a rare concurrence of primary immunodeficiency and celiac disease

Hypogammaglobulinemic sprue (HGGS) belongs to orphan (rare) diseases. Its basis is a concurrence of primary immunodeficiency and celiac disease. The clinical picture of HGGS is characterized by recurrent bacterial infection foci, chronic diarrhea, and severe malabsorption syndrome (MAS). Immunological changes are exhibited by a decrease in the serum levels of γ-globulins (GG), immunoglobulins (Ig) A and G, and, to a lesser extent, IgM and B lymphocytes. The peculiarity of the morphological pattern of HGGS is the atrophy of the gastric mucosa and small bowel mucosal villi, which is accompanied by lymphocytic infiltration, formation of lymphoid follicles in the proper mucosal plate, and a drastic drop in plasma cells. It is precisely these signs that serve as a basis for identifying HGGS as an independent nosological entity. Its management is to be on a life-long gluten-free diet (GFD), to administer GG or Ig monthly, to correct metabolic disturbances, and to treat focal infections. The paper describes a case history of a female patient with HGGS. The disease was characterized by recurrent lung and kidney inflammatory foci concurrent with chronic diarrhea and grade 3 MAS. There was a dramatic reduction in GG, IgG, and IgA levels. Clinical remission and small intestinal mucosal structural recovery occurred due to GFD.

common variable hypogammaglobulinemia, hypogammaglobulinemic sprue, celiac disease, gluten-free diet.