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). Immunologic...
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| Format: | Article |
| Language: | Russian |
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"Consilium Medicum" Publishing house
2014-12-01
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| Series: | Терапевтический архив |
| Subjects: | |
| Online Access: | https://ter-arkhiv.ru/0040-3660/article/view/31593 |
| _version_ | 1818923200605585408 |
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| author | A I Parfenov L M Krums R B Gudkova S G Khomeriki |
| author_facet | A I Parfenov L M Krums R B Gudkova S G Khomeriki |
| author_sort | A I Parfenov |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-12-20T02:05:39Z |
| format | Article |
| id | doaj.art-71a6fa01d7704b979da8f8dd6b38f211 |
| institution | Directory Open Access Journal |
| issn | 0040-3660 2309-5342 |
| language | Russian |
| last_indexed | 2024-12-20T02:05:39Z |
| publishDate | 2014-12-01 |
| publisher | "Consilium Medicum" Publishing house |
| record_format | Article |
| series | Терапевтический архив |
| spelling | doaj.art-71a6fa01d7704b979da8f8dd6b38f2112022-12-21T19:57:12Zrus"Consilium Medicum" Publishing houseТерапевтический архив0040-36602309-53422014-12-018612-2192128609Hypogammaglobulinemic sprue is a rare concurrence of primary immunodeficiency and celiac diseaseA I ParfenovL M KrumsR B GudkovaS G KhomerikiHypogammaglobulinemic 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.https://ter-arkhiv.ru/0040-3660/article/view/31593common variable hypogammaglobulinemiahypogammaglobulinemic sprueceliac diseasegluten-free diet. |
| spellingShingle | A I Parfenov L M Krums R B Gudkova S G Khomeriki Hypogammaglobulinemic sprue is a rare concurrence of primary immunodeficiency and celiac disease Терапевтический архив common variable hypogammaglobulinemia hypogammaglobulinemic sprue celiac disease gluten-free diet. |
| title | Hypogammaglobulinemic sprue is a rare concurrence of primary immunodeficiency and celiac disease |
| title_full | Hypogammaglobulinemic sprue is a rare concurrence of primary immunodeficiency and celiac disease |
| title_fullStr | Hypogammaglobulinemic sprue is a rare concurrence of primary immunodeficiency and celiac disease |
| title_full_unstemmed | Hypogammaglobulinemic sprue is a rare concurrence of primary immunodeficiency and celiac disease |
| title_short | Hypogammaglobulinemic sprue is a rare concurrence of primary immunodeficiency and celiac disease |
| title_sort | hypogammaglobulinemic sprue is a rare concurrence of primary immunodeficiency and celiac disease |
| topic | common variable hypogammaglobulinemia hypogammaglobulinemic sprue celiac disease gluten-free diet. |
| url | https://ter-arkhiv.ru/0040-3660/article/view/31593 |
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