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Year : 2008  |  Volume : 51  |  Issue : 2  |  Page : 307
Is positive c-ANCA mandatory for the diagnosis of Wegener's granulomatosis?

Department of Pathology, T. N. Medical College and B. Y. L. Ch Nair Hospital, Mumbai Central, Mumbai - 400 008, India

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How to cite this article:
Jashnani KD, Rupani AB, Deshpande JR, Karekar MD. Is positive c-ANCA mandatory for the diagnosis of Wegener's granulomatosis?. Indian J Pathol Microbiol 2008;51:307

How to cite this URL:
Jashnani KD, Rupani AB, Deshpande JR, Karekar MD. Is positive c-ANCA mandatory for the diagnosis of Wegener's granulomatosis?. Indian J Pathol Microbiol [serial online] 2008 [cited 2020 Jun 6];51:307. Available from: http://www.ijpmonline.org/text.asp?2008/51/2/307/41702


Diagnosis of Wegener's granulomatosis (WG) can be problematic because of its varied clinical presentations. [1] Detection of circulating c-antineutrophil cytoplasmic antibodies (c-ANCAs) in combination with histopathology permits one to identify WG at an early stage. We report a case diagnosed at autopsy where c-ANCA levels were not available to support the diagnosis.

A 45-year-old hypertensive and diabetic female presented with right otorrhoea, bilateral decreased hearing and intermittent otalgia of 2 years duration. There was a past history of nasal operation done 10 years back; details of which were not available. Blood urea nitrogen and serum creatinine levels were elevated along with oliguria and proteinuria. As the clinical impression was acute tubular necrosis, renal biopsy was not performed. Patient underwent modified radical mastoidectomy for otomastoiditis. Her aural complaints resolved and serum creatinine was also gradually coming to normal level. Patient suddenly expired and a complete autopsy was conducted in view of unexplained cause of death.

Grossly, organs like bilateral kidneys, spleen, liver and heart showed infarcts with no evidence of vegetations in the heart. Microscopic examination of both the kidneys, in addition to the infarcts, showed necrotizing glomerulonephritis with prominent periglomerular mixed inflammatory infiltrate and ill-defined granulomas. Eosinophils were not noted. Occasional glomeruli showed cellular crescents [Figure 1]. Small arterioles in the serosa of the ureter and the tissue from the right internal ear showed small vessel vasculitis. There were multiple arterial thrombi noted in the kidneys, lung and spleen. Heart, right adrenal gland and spleen showed fresh infarcts. A diagnosis of WG was made.

Anti-neutrophil cytoplasmic autoantibodies are useful serologic diagnostic markers for WG and positive in 80-90% of cases. In addition, one-third of patients with anti-GBM disease are ANCA positive. [1] Hence, the serologic markers are neither absolutely specific nor sensitive and thus, must be interpreted in the light of other available data. In the present case, c-ANCA levels were not available, as the diagnosis was not entertained clinically. If c-ANCA can be negative in 10-20% of the classic untreated WG and also be positive in 30% of anti-GBM disease, non-availability of c-ANCA levels cannot be used as a point against the diagnosis of WG, where all other clinical and histopathologic features are favourable, particularly at the autopsy. Silent myocardial infarction, splenic and adrenal infarcts are reported in WG in the published reports as seen in our case. [2],[3],[4]

At the otorhinolaryngology clinic, many cases of WG present with rhinitis, otitis media, facial palsy or laryngeal obstruction. [5]Patients with these presentations not responding to the standard treatment have to be further investigated for a diagnosis of WG. The prognosis of WG is good, if it is diagnosed early and treated with a combination of corticosteroids and cytotoxic drugs. Hence, we emphasize the importance of early diagnosis of WG with consideration of the systemic manifestations of this disease as seen in present case.

   References Top

1.Jennette JC. Renal involvement in systemic vasculitis. In: Heptinstall's Pathology of the Kidney. 5th ed. Vol 2. Philadelphia: Lippincott-Raven Publishers; 1998. p. 1059-95.  Back to cited text no. 1    
2.Lawson TM, Williams BD. Silent myocardial infarction in Wegener's granulomatosis. Br J Rheumatol 1996;35:188-91.  Back to cited text no. 2  [PUBMED]  [FULLTEXT]
3.Papaioannides D, Nikas SN, Fotinou M, Akritidis NK. Asymptomatic splenic infarction in Wegener's granulomatosis. Ann Rheum Dis 2002;61:185-6.  Back to cited text no. 3    
4.Thomas GO, Lewis RJ. Adrenal infarction: An unusual complication of Wegener's granulomatosis. Br J Dis Chest 1979;73: 178-80.  Back to cited text no. 4  [PUBMED]  
5.Xiao F, Lu W, Ni D, Yu H. Misdiagnosis analysis of Wegener's granulomatosis in otorhinolaryngology. Lin Chuang Er Bi Yan Hou Ke Za Zhi 2005;19:295- 6.  Back to cited text no. 5    

Correspondence Address:
Kusum D Jashnani
8, Ashirwad, 1st Floor, Opposite Kakad Industrial Estate, L. J. Cross Road-3, Mahim, Mumbai - 400 016
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.41702

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