Title |
IgG4- related disease: an orphan disease with many faces
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Published in |
Orphanet Journal of Rare Diseases, July 2014
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DOI | 10.1186/s13023-014-0110-z |
Pubmed ID | |
Authors |
Herwig Pieringer, Ilse Parzer, Adelheid Wöhrer, Petra Reis, Bastian Oppl, Jochen Zwerina |
Abstract |
Immunoglobulin G4- related disease (IgG4-RD) is a rare systemic fibro-inflammatory disorder (ORPHA284264). Although patients have been described more than 100 years ago, the systemic nature of this disease has been recognized in the 21st century only. Type 1 autoimmune pancreatitis is the most frequent manifestation of IgG4-RD. However, IgG4-RD can affect any organ such as salivary glands, orbits, retroperitoneum and many others. Recent research enabled a clear clinical and histopathological description of IgG4-RD. Typically, lymphoplasmacellular inflammation, storiform fibrosis and obliterative phlebitis are found in IgG4-RD biopsies and the tissue invading plasma cells largely produce IgG4. Elevated serum IgG4 levels are found in many but not all patients. Consequently, diagnostic criteria for IgG4-RD have been proposed recently. Treatment is largely based on clinical experience and retrospective case series. Glucocorticoids are the mainstay of therapy, although adjunctive immunosuppressive agents are used in relapsing patients. This review summarizes current knowledge on clinical manifestations, pathophysiology and treatment of IgG4-RD. |
X Demographics
Geographical breakdown
Country | Count | As % |
---|---|---|
France | 2 | 7% |
Ireland | 1 | 4% |
Japan | 1 | 4% |
Ecuador | 1 | 4% |
India | 1 | 4% |
Kuwait | 1 | 4% |
Germany | 1 | 4% |
Spain | 1 | 4% |
Canada | 1 | 4% |
Other | 2 | 7% |
Unknown | 16 | 57% |
Demographic breakdown
Type | Count | As % |
---|---|---|
Members of the public | 18 | 64% |
Scientists | 5 | 18% |
Practitioners (doctors, other healthcare professionals) | 5 | 18% |
Mendeley readers
Geographical breakdown
Country | Count | As % |
---|---|---|
United States | 1 | <1% |
Portugal | 1 | <1% |
South Africa | 1 | <1% |
Unknown | 137 | 98% |
Demographic breakdown
Readers by professional status | Count | As % |
---|---|---|
Other | 23 | 16% |
Student > Doctoral Student | 16 | 11% |
Researcher | 14 | 10% |
Student > Master | 14 | 10% |
Student > Ph. D. Student | 13 | 9% |
Other | 41 | 29% |
Unknown | 19 | 14% |
Readers by discipline | Count | As % |
---|---|---|
Medicine and Dentistry | 80 | 57% |
Agricultural and Biological Sciences | 9 | 6% |
Immunology and Microbiology | 6 | 4% |
Nursing and Health Professions | 6 | 4% |
Biochemistry, Genetics and Molecular Biology | 5 | 4% |
Other | 9 | 6% |
Unknown | 25 | 18% |