IgG4 disease

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Etiology

associated disorders

Epidemiology

  • male/female ratio: 2:1[14]

Pathology

Clinical manifestations

* case report of 36 year old woman presenting with pneumonia[5]

* case report of 57 year old woman[8]

Laboratory

Diagnostic procedures

Radiology

Complications

Management

More general terms

Additional terms

References

  1. 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://www.ncbi.nlm.nih.gov/pubmed/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://www.ncbi.nlm.nih.gov/pubmed/25477133 <Internet> http://ard.bmj.com/content/74/1/1
  2. 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.
  3. 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://www.ncbi.nlm.nih.gov/pubmed/24111912
  4. Stone JH, Zen Y, Deshpande V. IgG4-related disease. N Engl J Med. 2012 Feb 9;366(6):539-51 PMID: https://www.ncbi.nlm.nih.gov/pubmed/22316447
  5. 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://www.ncbi.nlm.nih.gov/pubmed/26510025
  6. 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/
  7. Saeki T, Kawano M. IgG4-related kidney disease. Kidney Int. 2014 Feb;85(2):251-7. PMID: https://www.ncbi.nlm.nih.gov/pubmed/24107849
  8. 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://www.ncbi.nlm.nih.gov/pubmed/2822568 <Internet> http://www.nejm.org/doi/full/10.1056/NEJMcpc1613461
  9. 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://www.ncbi.nlm.nih.gov/pubmed/23429355 Free PMC Article
  10. ARUP Consult IgG4-Related Disease - IgG4-RD https://arupconsult.com/content/igg4-related-disease
  11. Zhang P, Cornell LD. IgG4-Related Tubulointerstitial Nephritis. Adv Chronic Kidney Dis. 2017 Mar;24(2):94-100. Review. PMID: https://www.ncbi.nlm.nih.gov/pubmed/28284385
  12. de la Fuente J, Bird J Coronary Arteritis in IgG4-Related Disease. N Engl J Med 2019; 380:2156. May 30, 2019 PMID: https://www.ncbi.nlm.nih.gov/pubmed/31141636 https://www.nejm.org/doi/full/10.1056/NEJMicm1809588
  13. 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://www.ncbi.nlm.nih.gov/pubmed/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://www.ncbi.nlm.nih.gov/pubmed/33274670
  14. 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://www.ncbi.nlm.nih.gov/pubmed/38824935 PMCID: PMC11214762 (available on 2025-07-01) https://www.thelancet.com/journals/lanrhe/article/PIIS2665-9913(24)00089-4/abstract

Patient information

IgG4 disease patient information