Clinical Case Database / Category: Patient Management

A case of granulomatosis with polyangiitis

Publication details

Dr Amanda Isdale MBChB, MRCP, Dr Marilyn Rogers MD, MRCP(Edin.)
Foundation Years Journal, volume 9, issue 10, p.6 (123Doc Education, London, November 2015)

Abstract

A 57 year old lady presented with a 3 month history of breathlessness, non-resolving consolidation on chest X-ray, lacrimal gland enlargement, ptosis and orbital pain. Blood investigations showed leucocytosis, thrombocytosis, a normocytic anaemia, elevated C-reactive protein (CRP), normal renal function and urinalysis, and negative anti-neutrophil cytoplasmic antibody (ANCA). A malignancy was suspected, but only after extensive investigations were other diagnoses re-considered. Subsequent biopsy from the lacrimal gland demonstrated granulomatous inflammation with vasculitis and necrosis, consistent with a diagnosis of granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis. It is important to consider vasculitis as a possible diagnosis in patients with multisystem presentations even when ANCA is negative, as in this case, as delays in diagnosis and treatment may have organ or life- threatening consequences.

Access the Clinical Cases Database

A subscription is required to read the full article. Please subscribe using one of the options below.

Authors

Dr Amanda Isdale MBChB, MRCP (Corresponding author)

Consultant Rheumatologist
York Teaching Hospital NHS Foundation Trust
York Hospital
Wigginton Road
York
YO31 8HE
amanda.isdale@york.nhs.uk

Dr Marilyn Rogers MD, MRCP(Edin.)

Specialist Registrar in Rheumatology
York Teaching Hospital NHS Foundation Trust
York Hospital
Wigginton Road
York
YO31 8HE
marilyn.rogers@york.nhs.uk

References

1. Tracy CL, Papadopoulos PJ. Granulomatosis with Polyangiitis (Wegener Granulomatosis) Workup. [Online] Available from: http://emedicine.medscape.com/article/332622-workup#showall [Accessed 10th July 2015]
2. Booth AD, Almond MK, Burns A, et al. Outcome of ANCA-associated renal vascultis: a 5-year retrospective study. Am J Kidney Dis 2003; 41:776-784.
3. Westman KW, Bygren PG, Olsson H, et al. Relapse rate, renal survival, and cancer morbidity in patients with Wegener's granulomatosis or microscopic polyangiitis with renal involvement. J Am Soc Nephrol 1998; 9:842-852.
4. Finkielman JD, Lee AS, Hummel AM et. Al. WGET Research Group. ANCA are detectable in nearly all patients with active severe Wegener's granulomatosis. Am J Med. 2007; 120(7):643.e9.
5. Walton EW. Giant-cell granuloma of the respiratory tract (Wegener's granulomatosis). Br Med J. 1958 Aug 2;2(5091):265–270.
6. Phillip R, Luqmani R. Mortality in systemic vasculitis: a systematic review. Clin Exp Rheumatol. 2008. 26:S94-S104.
7. Wung PK, Stone JH. Therapeutics of wegener's granulomatosis. Nature Clinical Practice Rheumatology. 2006; 2:192-200.
8. Faurschou M, Mellemkjaer L, Voss A, Keller KK, Hansen IT, Baslund B. Prolonged risk of specific malignancies following cyclophosphamide therapy among patients with granulomatosis with polyangiitis. Rheumatology. 2015; 54:1345-1350.
9. Ntatsaki E, Carruthers D, Chakravarty K et. al BSR and BHPR Standards, Guidelines and Audit Working Group. BSR and BHPR guideline for the management of adults with ANCA-associated vasculitis. [Online] Available from: http://www.rheumatology.org.uk/includes/documents/cm_docs/2014/2/2014vasculitisfull_guidelines_new.pdf [Accessed 18th July 2015]

Disclaimers

Conflict Of Interest

The Journal requires that authors disclose any potential conflict of interest that they may have. This is clearly stated in the Journal’s published “Guidelines for Authors”. The Journal follows the Guidelines against Conflict of Interest published in the Uniform Requirements for Manuscripts Submitted to Biomedical Journals (http://www.icmje.org/urm_full.pdf).

Financial Statement

The authors of this article have not been paid. The Journal is financed by subscriptions and advertising. The Journal does not receive money from any other sources. The decision to accept or refuse this article for publication was free from financial considerations and was solely the responsibility of the Editorial Panel and Editor-in-Chief.

Patient Consent statement

All pictures and investigations shown in this article are shown with the patients’ consent. We require Authors to maintain patients’ anonymity and to obtain consent to report investigations and pictures involving human subjects when anonymity may be compromised. The Journal follows the Guidelines of the Uniform Requirements for Manuscripts (http://www.icmje.org/urm_full.pdf). The Journal requires in its Guidelines for Authors a statement from Authors that “the subject gave informed consent”.

Animal & Human Rights

When reporting experiments on human subjects, the Journal requires authors to indicate whether the procedures followed were in accordance with the ethical standards of the responsible committee on human experimentation (institutional and national) and with the HelsinkiDeclaration of 1975, as revised in 2008.

About the Clinical Cases Database

T​he Foundation Years Clinical Cases Database is​ a selection of 600 peer-reviewed clinical cases in the field of patient safety and clinical practice, specifically focused on the clinical information needs of junior doctors, based around the Foundation Year Curriculum programme (MMC). The cases have been chosen to align with the Foundation Year Curriculum.

The database is fully searchable, or can be browsed by medical specialty. Abstracts can be read free of charge, however a subscription is required in order to read the complete cases.