Abstract
The standard description of Wegener's granulomatosis emphasises renal failure and thus a distorted impression may be given. Subacute and even chronic cases occur, and in these patients the presentation is varied and often insidious, leading to delay in diagnosis. Twenty two such patients (13 women and nine men) with a mean age of 44 years were seen in our connective tissue disease clinic. The mean duration of symptoms before diagnosis was 3.6 years and the mean duration of disease 5.9 years (19 years in one patient). All patients had malaise and ear, nose, and throat symptoms, and most had joint pains. Impaired renal function was seen in seven patients only. Tissue biopsy was diagnostic in half of the patients, and appreciably high titres of antineutrophil cytoplasmic antibodies were detected in only nine of 18 patients in whom these were measured. The most useful investigations were neutrophil counts, chest radiographs, and computed tomography of the sinuses and orbits. The most effective treatment was with intravenous pulses of cyclophosphamide. No deaths occurred. At the time of writing two patients were in remission and no longer being treated and 18 patients were in partial remission on continued treatment. Patients with subacute forms of Wegener's granulomatosis present with a variety of clinical features and the insidious presentation often leads to delay in diagnosis. A history of ear, nose, and throat symptoms was universal in our patients.
Full text
PDF
Images in this article
Selected References
These references are in PubMed. This may not be the complete list of references from this article.
- Carrington C. B., Liebow A. Limited forms of angiitis and granulomatosis of Wegener's type. Am J Med. 1966 Oct;41(4):497–527. doi: 10.1016/0002-9343(66)90214-2. [DOI] [PubMed] [Google Scholar]
- Coutu R. E., Klein M., Lessell S., Friedman E., Snider G. L. Limited form of Wegener granulomatosis. Eye involvement as a major sign. JAMA. 1975 Aug 25;233(8):868–871. [PubMed] [Google Scholar]
- FAHEY J. L., LEONARD E., CHURG J., GODMAN G. Wegener's granulomatosis. Am J Med. 1954 Aug;17(2):168–179. doi: 10.1016/0002-9343(54)90255-7. [DOI] [PubMed] [Google Scholar]
- Fauci A. S., Haynes B. F., Katz P., Wolff S. M. Wegener's granulomatosis: prospective clinical and therapeutic experience with 85 patients for 21 years. Ann Intern Med. 1983 Jan;98(1):76–85. doi: 10.7326/0003-4819-98-1-76. [DOI] [PubMed] [Google Scholar]
- Lanham J. G., Elkon K. B., Pusey C. D., Hughes G. R. Systemic vasculitis with asthma and eosinophilia: a clinical approach to the Churg-Strauss syndrome. Medicine (Baltimore) 1984 Mar;63(2):65–81. doi: 10.1097/00005792-198403000-00001. [DOI] [PubMed] [Google Scholar]
- Lockwood C. M., Bakes D., Jones S., Whitaker K. B., Moss D. W., Savage C. O. Association of alkaline phosphatase with an autoantigen recognised by circulating anti-neutrophil antibodies in systemic vasculitis. Lancet. 1987 Mar 28;1(8535):716–720. doi: 10.1016/s0140-6736(87)90357-6. [DOI] [PubMed] [Google Scholar]
- Noritake D. T., Weiner S. R., Bassett L. W., Paulus H. E., Weisbart R. Rheumatic manifestations of Wegener's granulomatosis. J Rheumatol. 1987 Oct;14(5):949–951. [PubMed] [Google Scholar]
- Pinching A. J., Lockwood C. M., Pussell B. A., Rees A. J., Sweny P., Evans D. J., Bowley N., Peters D. K. Wegener's granulomatosis: observations on 18 patients with severe renal disease. Q J Med. 1983 Autumn;52(208):435–460. [PubMed] [Google Scholar]
- Reza M. J., Dornfeld L., Goldberg L. S., Bluestone R., Pearson C. M. Wegener's granulomatosis. Long-term followup of patients treated with cyclophosphamide. Arthritis Rheum. 1975 Sep-Oct;18(5):501–506. doi: 10.1002/art.1780180513. [DOI] [PubMed] [Google Scholar]
- Ridley M. G., Wolfe C. S., Asherson R. A., Hughes G. R. Recurrent otitis media and pulmonary infiltrates as presenting features in Wegener's granulomatosis. BMJ. 1988 Jul 30;297(6644):352–353. doi: 10.1136/bmj.297.6644.352. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Savage C. O., Winearls C. G., Jones S., Marshall P. D., Lockwood C. M. Prospective study of radioimmunoassay for antibodies against neutrophil cytoplasm in diagnosis of systemic vasculitis. Lancet. 1987 Jun 20;1(8547):1389–1393. doi: 10.1016/s0140-6736(87)90591-5. [DOI] [PubMed] [Google Scholar]
- Spalton D. J., Graham E. M., Page N. G., Sanders M. D. Ocular changes in limited forms of Wegener's granulomatosis. Br J Ophthalmol. 1981 Aug;65(8):553–563. doi: 10.1136/bjo.65.8.553. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Wolff S. M., Fauci A. S., Horn R. G., Dale D. C. Wegener's granulomatosis. Ann Intern Med. 1974 Oct;81(4):513–525. doi: 10.7326/0003-4819-81-4-513. [DOI] [PubMed] [Google Scholar]