The usefulness of histopathological examinations of non-renal biopsies in the diagnosis of granulomatosis with polyangiitis

Abstract

IntroductionGranulomatosis with polyangiitis (GPA) is a rare, ANCA-associated, systemic disease characterized by necrotizing small and medium vessel vasculitis of unknown etiology associated with granulomatous inflammation affecting the renal, pulmonary, upper airways, ocular systems and other tissues. Histological proof of the granulomatosis with polyangiitis (GPA) can be obtained by biopsy of clinically involved sites. The main purpose of this study was to examine histopathological changes in non-renal biopsies from patients with established diagnosis of GPA and evaluated the histological confirmation at diagnosis of this disease.Material and methodsA retrospective analysis was performed in patients with GPA diagnosed and treated in clinics of the University Clinical Center (UCK) in Gdansk in 1988–2009.ResultsIn the analyzed group of GPA patients the histopathological examination of biopsies taken from involved tissues (except kidney) was performed in 60% of patients. Thirty-six out of 93 biopsies (39%) were diagnosed as typical of GPA, 10 (10.7%) were suggestive and 51 (54.8%) were non-specific. Considering all biopsies, the diagnosis was confirmed in 24 patients (57% of patients in whom biopsies were taken). Epitheloid cell granulomas were present in 33 biopsies (43%), characteristic necrosis in 27 biopsies (35%), small vessel vasculitis in 18 biopsies (23%), while multinucleated giant cells were identified only in 9 biopsies (12%).ConclusionsHistopathological examination of the affected tissues remains the gold standard of the diagnosis of GPA. Its usefulness increases, particularly in ANCA-negative patients, in the initial phase of the disease, or in patients with atypical clinical presentation. In many cases, it is necessary to repeat biopsy to establish the diagnosis. The role of the histopathological examination seems to be particularly important when ANCA is negative or clinical symptoms are atypical of GPA.

A case of ANCA-associated vasculitis in a 16-year-old female following SARS-COV-2 infection and a systematic review of the literature

“…The gold standard for diagnosing childhood vasculitis is with either histopathology from tissue biopsy or by characteristic lesions detected by imaging studies. Multiple studies in adults with AAV have found that less than half of all biopsies show the characteristic inflammatory and vasculitic changes to confirm the diagnosis [ 15 – 17 ], and sampling difficulty is often a limitation which could have explained our patient’s biopsy findings. Different classification criteria have been proposed to diagnose GPA in children [ 5 , 18 – 20 ].…”

Rapidly recurrent prostatic obstruction due to granulomatosis with polyangiitis

“… 1 Pulmonary biopsy has a sensitivity ranging from 10 to 80% while the sensitivity of biopsies from the nose or sinuses is estimated at 20–50%. 5 …”

Focal proliferative and necrotizing glomerulonephritis presenting with mild urinary abnormalities and normal renal function in a young adult

“…In addition, a biopsy of extra renal tissue has a rather low yield for a definitive diagnosis of ANCA-related vasculitis, and in many cases, it is necessary to repeat biopsy and make repeated attempts to establish the diagnosis. 10…”