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The Classification & Diagnosis of Granulomatosis with Polyangiitis

Harry E. Subramanian, Ravi Sutaria, MD, & Fotios Koumpouras, MD  |  Issue: August 2018  |  August 16, 2018

Diagnostic difficulty can lead to a delay in initiation of the proper treatment, and a thorough evaluation of the patient is required to determine the final diagnosis and correct management in a timely manner.


Harry SubramanianHarry Subramanian is a medical student at the Yale School of Medicine. He will be pursuing a residency in diagnostic radiology at the Yale-New Haven Hospital.

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Ravi B. Sutaria, MDRavi B. Sutaria, MD, is a graduating fellow of Rheumatology at the Yale School of Medicine. Prior to joining Yale he was a clinical instructor of medicine at Jacobi Medical Center at North Central Bronx Hospital in New York during his time as a hospitalist. He is pursuing research establishing the efficacy of DMARDs in the treatment of chronic rheumatism as result of emerging viral infections, such as chikungunya. He is also interested in interventional/diagnostic musculoskeletal ultrasound, particularly in patients with autoimmune and inflammatory disorders.

Fotios Koumpouras, MDFotios Koumpouras, MD, is assistant professor of medicine and director of the Yale Lupus Program. He is an active member of the ACR, the Arthritis Foundation and the Lupus Foundation. In addition to SLE, Dr. Koumpouras has an interest in lupus biomarkers, pregnancy in rheumatic disease, psoriatic arthritis, diagnostic and treatment dilemmas, adolescent arthritis, lupus and the heart, gout and the gender gap of autoimmune disorders.

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Disclosures

None of the authors reports any rele­vant disclosures for this project, but Dr. Koumpouras reports he has previously received grant funding from NIH, NIAMS and Yale University and conducted clinical trials with Merck, Xencor, GSK, Aurinia Pharmaceuticals, UCB and the Lupus Clinical Investigators Network.

References

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  2. Lutalo PM, D’Cruz DP. Diagnosis and classification of granulomatosis with polyangiitis (aka Wegener’s granulomatosis). J Autoimmun. 2014;48–49:94–98.
  3. Comarmond C, Cacoub P. Granulomatosis with polyangiitis (Wegener): Clinical aspects and treatment. Autoimmun Rev. 2014 Nov;13(11):1121–1125.
  4. Grant SC, Levy RD, Venning MC, et al. Wegener’s granulomatosis and the heart. Br Heart J. 1994 Jan;71(1):82–86.
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  6. Weiner M, Segelmark M. The clinical presentation and therapy of diseases related to anti-neutrophil cytoplasmic antibodies (ANCA). Autoimmun Rev. 2016 Oct;15(10):978–982.
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  8. Li JS, Sexton DJ, Mick N, et al. Proposed modifications to the Duke criteria for the diagnosis of infective endocarditis. Clin Infect Dis. 2000 Apr;30(4):633–638.
  9. Leavitt RY, Fauci AS, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of Wegener’s granulomatosis. Arthritis Rheum. 1990;33(8):1101–1107.
  10. Jennette JC, Falk RJ. Small-vessel vasculitis. N Engl J Med. 1997 Nov 20;337(21):1512–1523.
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  12. McGeoch L, Carette S, Cuthbertson D, et al. Cardiac involvement in granulomatosis with polyangiitis. J Rheumatol. 2015 Jul;42(7):1209–1212.
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  14. Lacoste C, Mansencal N, Ben M’rad M, et al. Valvular involvement in ANCA-associated systemic vasculitis: A case report and literature review. BMC Musculoskelet Disord. 2011 Feb 23;12:50.
  15. Anthony DD, Askari AD, Wolpaw T, McComsey G. Wegener granulomatosis simulating bacterial endocarditis. Arch Intern Med. 1999 Aug 9–23;159(15):1807–1810.
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  18. Mishell JM. Cases from the Osler Medical Service at Johns Hopkins University: Cardiac valvular lesions in Wegener’s granulamatosis. Am J Med. 2002 Nov;113(7):607–609.
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