ReviewHaematologica2019
IgG4-related disease: what a hematologist needs to know.
Review in Haematologica, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 91 papers, 1 of them a synthesis that pooled it.
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Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
91 citing papers in PubMed, 1 synthesis or guideline pooled it, 191 citations in OpenAlex.
- IgG4-related disease with multiorgan involvement: a case-based review.Rheumatology international · 2021Pooled it
- A case report: an unusual case of ulcerative intestinal IgG4-related disease with clinical features overlapping with probable intestinal Behcet's disease.BMC gastroenterology · 2026Article
- Midline Destructive Lesions of Face: A Dermatology Perspective.Indian dermatology online journal · 2026Article
- The Utility of IgG4/IgG Ratio in the Diagnosis of Multicentric Castleman Disease: A Case Report of HHV8+ Castleman Disease in a Patient with Classical Hodgkin's Lymphoma.Hematology reports · 2026Article
- Immunoglobulin-G4 Related Disease: A Rare Entity with Many Clinical Faces - Literature Review and Case Illustration.Mediterranean journal of rheumatology · 2026Review
- Azathioprine as maintenance therapy for IgG4-related diseases: a retrospective case series and case-based review of the literature.Rheumatology international · 2026Review
- Immunological Biomarkers to Assess Activity and Treatment Response in IgG4-Related Disease.Medicina (Kaunas, Lithuania) · 2026Article
- Case Report: A case of primary IgG4-related skin disease presenting initially with skin papules and abscesses.Frontiers in immunology · 2026Article
- Correlations between IgG4-related disease, autoimmune pancreatitis, and allergic diseases.Frontiers in immunology · 2026Review
- Case Report: Serum IgG4-negative multifocal IgG4-related hepatic inflammatory pseudotumor: dissociation between circulating IgG4 and local immune activation.Frontiers in immunology · 2026Article
- Case Report: IgG4-related autoimmune pancreatitis presenting as an infiltrative cystic-solid pancreatic mass: lessons from a diagnostic pitfall.Frontiers in medicine · 2026Article
- Article
- IgG4-Related Disease: Emerging Roles of Novel Genetic Variants, Immune Cell Subsets and Therapeutic Targets.Allergy · 2025Review
- IgG4-Related Orbital Disease vs. Idiopathic Orbital Inflammation: Clinical Features, Therapy and Outcomes in a Central-European Retrospective Single-Center Cohort.Biomedicines · 2025Article
- Successful treatment of Kimura disease with Mycophenolate Mofetil: a report of two cases and review of the literature.Rheumatology international · 2025Review
- Pulmonary plasma cell disorders: histopathology, diagnosis, and clinical perspectives.Virchows Archiv : an international journal of pathology · 2025Review
- IgG4-Related Disease: Current and Future Insights into Pathological Diagnosis.International journal of molecular sciences · 2025Review
- CT surveillance for type 1 autoimmune pancreatitis: cumulative radiation dose and diagnostic performance for disease relapse.European radiology · 2025Article
- Article
- A Rare Presentation of a Spinal Lesion: Immunoglobulin G4-Related Hypertrophic Spinal Pachymeningitis.Cureus · 2025Article
31 more citing papers are in PubMed but not listed here.
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
4 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
IgG4-related disease is a fibro-inflammatory condition that can affect nearly any organ system. Common presentations include major salivary and lacrimal gland enlargement, orbital disease, autoimmune pancreatitis, retroperitoneal fibrosis and tubulointerstitial nephritis. This review focuses on the hematologic manifestations of IgG4-related disease, including lymphadenopathy, eosinophilia, and polyclonal hypergammaglobulinemia. The disease can easily be missed by unsuspecting hematologists, as patients may present with clinical problems that mimic disorders such as multicentric Castleman disease, lymphoma, plasma cell neoplasms and hypereosinophilic syndromes. When IgG4-related disease is suspected, serum protein electrophoresis and IgG subclasses are helpful as initial tests but a firm histological diagnosis is essential both to confirm the diagnosis and to rule out mimickers. The central histopathological features are a dense, polyclonal, lymphoplasmacytic infiltrate enriched with IgG4-positive plasma cells (with an IgG4/IgG ratio >40%), storiform fibrosis, and obliterative phlebitis. Importantly for hematologists, the latter two features are seen in all tissues except bone marrow and lymph nodes, making these two sites suboptimal for histological confirmation. Many patients follow an indolent course and respond well to treatment, but a significant proportion may have highly morbid or fatal complications such as periaortitis, severe retroperitoneal fibrosis or pachymeningitis. Corticosteroids are effective but cause new or worsening diabetes in about 40% of patients. Initial response rates to rituximab are high but durable remissions are rare. More intensive lymphoma chemotherapy regimens may be required in rare cases of severe, refractory disease, and targeted therapy against plasmablasts, IgE and other disease biomarkers warrant further exploration.
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Registered trials
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.