ArticleOrphanet journal of rare diseases2014
IgG4- related disease: an orphan disease with many faces.
Article in Orphanet journal of rare diseases, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 51 papers, 1 of them a synthesis that pooled it.
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Who cites it
51 citing papers in PubMed, 1 synthesis or guideline pooled it.
- IgG4-related disease: a systematic review of this unrecognized disease in pediatrics.Pediatric rheumatology online journal · 2016Pooled it
- IgG4-related lung disease mimicking COPD: case report and review.BMC pulmonary medicine · 2025Review
- Symmetrical polyarthritis in IgG4-related sialadenitis: a diagnostic challenge with seronegative rheumatoid arthritis.BMC rheumatology · 2025Article
- Coexistence of IgG4-related disease and ANCA-associated vasculitis: case report and review of the literature.Rheumatology international · 2024Review
- Coexistence of Colorectal Cancer and Immunoglobulin G4-Related Disease in the Same Lesion: A Rare Case with Molecular Classification.Diagnostics (Basel, Switzerland) · 2024Article
- Renal Manifestations of IgG4-Related Disease: A Concise Review.International journal of nephrology · 2024Review
- IgG4-related digestive diseases: diagnosis and treatment.Frontiers in immunology · 2023Review
- The Pancreas and Known Factors of Acute Pancreatitis.Journal of clinical medicine · 2022Review
- Diagnosis and Treatment of Acute Pancreatitis.Diagnostics (Basel, Switzerland) · 2022Review
- Article
- Case Report: Diagnosis of Petrous Apex IgG4-Related Disease by Middle Cranial Fossa Craniotomy and Temporal Bone Biopsy.Frontiers in neurology · 2022Article
- T Cell Roles and Activity in Chronic Sclerosing Sialadenitis as IgG4-Related Disease: Current Concepts in Immunopathogenesis.Autoimmune diseases · 2022Review
- Colonic Mucosal Infiltration of IgG4 Plasma Cells and Ulcerative Colitis: Determinant of Presence, Activity, Extension, and Duration of Disease.Middle East journal of digestive diseases · 2021Article
- A unique case of IgG4-related skin disease and sclerosing cholangitis in a patient with previous hepatitis exposure.JAAD case reports · 2021Article
- A pheochromocytoma wrapped in an IgG4-related disease.European journal of nuclear medicine and molecular imaging · 2021Article
- IgG4-Related Laryngeal Disease as a Possible Cause of Idiopathic Subglottic Stenosis: A Case Report.Iranian journal of otorhinolaryngology · 2021Article
- IgG4-Related Disease with Selective Testicular Involvement- A Rare Entity: Case Report with Review of Literature.Turk patoloji dergisi · 2021Review
- Hyper IgG4-Related Disease Presenting with Orbital Tumor and Immune Deficiency.Case reports in immunology · 2021Article
- Multi-organ IgG4-related disease continues to mislead clinicians: A case report and literature review.World journal of clinical cases · 2020Article
- Immunoglobulin G4 Related Lung Disease.Turkish thoracic journal · 2020Article
Corrections and comments
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Authors and funding
6 authors.
Funding
No grant is acknowledged in the PubMed record.
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.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.