IgG4 disease
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Etiology
associated disorders
- hypertrophic pachymeningitis
- lymphocytic hypophysitis
- Mikulicz syndrome
- dacryoadenitis
- Kuttner tumor
- Riedel thyroiditis
- aortitis
- autoimmune pancreatitis (recurrent pancreatitis)
- retroperitoneal fibrosis (Ormond disease)
- tubulointerstitial nephropathy
- sclerosing cholangitis
Epidemiology
- male/female ratio: 2:1[14]
Pathology
- biopsy (other than lymph node)
- dense lymphocytic infiltrate
- abundant IgG4 producing plasma cells in tissue
- storiform fibrosis
- obliterative phlebitis
- organ enlargement, fibrosis, dysfunction
- mild tissue eosinophilia
- increased numbers of IgG4-positive plasma cells
- IgG4 antibodies are generally regarded as noninflammatory; there is no evidence that they are directly pathogenic[3]
Clinical manifestations
- inflammation & enlargement of
- pancreas (autoimmune pancreatitis) thus abdominal pain
- lymph nodes (lymphadenopathy)
- salivary glands
- Mikulicz syndrome
- salivary gland inflammation & enlargement, xerostomia
- sclerosing sialadenitis
- parotid gland enlargement, parotitis
- lacrimal gland inflammation (dacryoadenitis)
- Kuttner tumor (submandibular glands)
- salivary gland inflammation & enlargement, xerostomia
- Mikulicz syndrome
- kidney (tubulointerstitial nephropathy)
- inflammation of periaortic region (idiopathic aortitis) leading to retroperitoneal fibrosis
- retroperitoneal involvement in 20%[6]
- dermatitis (rash, skin lesions)
- hypertrophic pachymeningitis (headache)
- lymphocytic hypophysitis
- Riedel thyroiditis[2]
- orbital inflammatory mass (orbital pseudotumor)
- sclerosing cholangitis
- lung & pleural involvement in 15-54%[6]
- clinical consequences of hypertrophy & mass effect of organ enlargement, pseudotumor, adenopathy ...
- night sweats, weight loss[8]
* case report of 36 year old woman presenting with pneumonia[5]
* case report of 57 year old woman[8]
Laboratory
- IgG4 in serum increased in 70% of cases[2]
- staining for IgG4 producing plasma cells
- serum IgE may be elevated
- complete blood count
Diagnostic procedures
- CT-guided biopsy with immunohistochemical staining for plasma cells & immunoglobulins (see Laboratory:)
Radiology
Complications
- periaortic mass lesion obstructing ureters
Management
- prednisone for autoimmune pancreatitis & sclerosing cholangitis[2]
- rituximab[3]
- inebilizumab-cdon (Uplizna)*
* FDA-approved
More general terms
Additional terms
References
- ↑ Carruthers MN et al. The diagnostic utility of serum IgG4 concentrations in IgG4- related disease. Ann Rheum Dis 2015 Jan; 74:14. <PubMed> PMID: https://pubmed.ncbi.nlm.nih.gov/24651618 <Internet> http://ard.bmj.com/content/74/1/14
Fox RI, Fox CM. IgG4 levels and plasmablasts as a marker for IgG4-related disease (IgG4-RD). Ann Rheum Dis 2015 Jan; 74:1. <PubMed> PMID: https://pubmed.ncbi.nlm.nih.gov/25477133 <Internet> http://ard.bmj.com/content/74/1/1 - ↑ 2.0 2.1 2.2 2.3 2.4 Medical Knowledge Self Assessment Program (MKSAP) 17, 18. American College of Physicians, Philadelphia 2015, 2018.
Medical Knowledge Self Assessment Program (MKSAP) 20 American College of Physicians, Philadelphia 2025 - ↑ 3.0 3.1 3.2 Mahajan VS, Mattoo H, Deshpande V, Pillai SS, Stone JH. IgG4-related disease. Annu Rev Pathol. 2014;9:315-47 PMID: https://pubmed.ncbi.nlm.nih.gov/24111912
- ↑ Stone JH, Zen Y, Deshpande V. IgG4-related disease. N Engl J Med. 2012 Feb 9;366(6):539-51 PMID: https://pubmed.ncbi.nlm.nih.gov/22316447
- ↑ 5.0 5.1 Khosroshahi A, Digumarthy SR, Gibbons FK, Deshpande V. CASE RECORDS of the MASSACHUSETTS GENERAL HOSPITAL. Case 34-2015. A 36-Year-Old Woman with a Lung Mass, Pleural Effusion, and Hip Pain. N Engl J Med. 2015 Oct 29;373(18):1762-72 PMID: https://pubmed.ncbi.nlm.nih.gov/26510025
- ↑ 6.0 6.1 6.2 Rothaus C A Woman with a Lung Mass. Now@NEJM. October 30th, 2015 http://blogs.nejm.org/now/index.php/a-woman-with-a-lung-mass/2015/10/30/
- ↑ Saeki T, Kawano M. IgG4-related kidney disease. Kidney Int. 2014 Feb;85(2):251-7. PMID: https://pubmed.ncbi.nlm.nih.gov/24107849
- ↑ 8.0 8.1 8.2 Monach PA, Stone JH, Sharma A, Nazarian RM. Case 6-2017 - A 57-Year-Old Woman with Fatigue, Sweats, Weight Loss, Headache, and Skin Lesions. N Engl J Med 2017; 376:775-786. February 23, 2017 <PubMed> PMID: https://pubmed.ncbi.nlm.nih.gov/2822568 <Internet> http://www.nejm.org/doi/full/10.1056/NEJMcpc1613461
- ↑ Khosroshahi A, Carruthers MN, Stone JH et al Rethinking Ormond's disease: "idiopathic" retroperitoneal fibrosis in the era of IgG4-related disease. Medicine (Baltimore). 2013 Mar;92(2):82-91. PMID: https://pubmed.ncbi.nlm.nih.gov/23429355 Free PMC Article
- ↑ ARUP Consult IgG4-Related Disease - IgG4-RD https://arupconsult.com/content/igg4-related-disease
- ↑ Zhang P, Cornell LD. IgG4-Related Tubulointerstitial Nephritis. Adv Chronic Kidney Dis. 2017 Mar;24(2):94-100. Review. PMID: https://pubmed.ncbi.nlm.nih.gov/28284385
- ↑ de la Fuente J, Bird J Coronary Arteritis in IgG4-Related Disease. N Engl J Med 2019; 380:2156. May 30, 2019 PMID: https://pubmed.ncbi.nlm.nih.gov/31141636 https://www.nejm.org/doi/full/10.1056/NEJMicm1809588
- ↑ NEJM Knowledge+ Complex Medical Care
Khosroshahi A, Wallace ZS, Crowe JL et al International Consensus Guidance Statement on the Management and Treatment of IgG4-Related Disease. Arthritis Rheumatol. 2015 Jul;67(7):1688-99. PMID: https://pubmed.ncbi.nlm.nih.gov/25809420 Review.
Umehara H, Okazaki K, Kawa S et al Research Program for Intractable Disease by the Ministry of Health, Labor and Welfare (MHLW) Japan. The 2020 revised comprehensive diagnostic (RCD) criteria for IgG4-RD. Mod Rheumatol. 2021 May;31(3):529-533. PMID: https://pubmed.ncbi.nlm.nih.gov/33274670 - ↑ 14.0 14.1 Jha I, McMahon GA, Perugino CA et al Sex as a predictor of clinical phenotype and determinant of immune response in IgG4-related disease: a retrospective study of patients fulfilling the American College of Rheumatology-European League Against Rheumatism classification criteria. Lancet Rheumatology. 2024 May 30 PMID: https://pubmed.ncbi.nlm.nih.gov/38824935 PMCID: PMC11214762 (available on 2025-07-01) https://www.thelancet.com/journals/lanrhe/article/PIIS2665-9913(24)00089-4/abstract
- ↑ Uchida K, Okazaki K. Current status of type 1 (IgG4-related) autoimmune pancreatitis. J Gastroenterol. 2022;57:695-708. PMID: https://pubmed.ncbi.nlm.nih.gov/35916965