The pulmonary biopsy in the early diagnosis of Wegener's (pathergic) granulomatosis: A study based on 35 open lung biopsies

Mark, Eugene J.; Matsubara, Osamu; Tan-Liu, N.; Fienberg, Robert

doi:10.1016/s0046-8177(88)80088-1

Cited by 142 publications

(86 citation statements)

References 27 publications

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“…A wide spectrum of tissue changes is known to occur in WG, and histopathological diagnosis, like clinical diagnosis, relies on a combination of findings rather than on any single feature [1,18,19]. The main criteria for WG are necrotizing vasculitis involving predominantly small arteries and veins, granulomata consisting of histiocytes and multinucleated giant cells, and "geographic" necrosis [1,18,19].…”

Section: Discussionmentioning

confidence: 99%

Efficacy of transbronchial biopsy in pulmonary vaculitides

Schnabel¹,

Holl‐Ulrich²,

Dalhoff³

et al. 1997

Eur Respir J

122

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This study was performed to determine the value of transbronchial biopsy (TBB) in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides and mild-to-moderate pulmonary involvement. Included in the study were 19 patients with Wegener's granulomatosis (WG) and six patients with Churg-Strauss syndrome (CSS) with evidence of active pulmonary disease but without gross parenchymal lesions accessible by radiologically guided biopsy. All of the patients had undergone staging examinations which included TBB taken from peripheral lung tissue and from any focal tracheobronchial lesions. Any suspicious lesion in the upper respiratory tract was biopsied by an otolaryngologist and the number of positive biopsies was compared with that of TBB. In the WG patients, only two out of 17 biopsies of alveolar tissue yielded histopathological findings supporting the diagnosis of WG. In five WG patients, ulcerative or exophytic airway lesions were found whose histopathologies were invariably positive. Otolaryngological examination revealed abnormal findings in 19 WG patients and biopsies from these sites yielded positive results in 13 instances. In CSS, TBB produced a diagnostically helpful histopathology in four of six cases and biopsies from the upper respiratory tract were positive in five out of six cases. We conclude that transbronchial biopsies of alveolar tissue are seldom positive in Wegener's granulomatosis patients with mild-to-moderate pulmonary disease unless they are taken from grossly abnormal lung areas. Conversely, ulcerative, exophytic or stenotic tracheobronchial lesions had a high rate of positive findings. These results further suggest that the upper rather than the lower respiratory tract should be the biopsy site of first choice in Wegener's granulomatosis. In Churg-Strauss syndrome, the upper and lower respiratory tract seem to yield a roughly equal number of positive biopsies.

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Section: Discussionmentioning

confidence: 99%

Efficacy of transbronchial biopsy in pulmonary vaculitides

Schnabel¹,

Holl‐Ulrich²,

Dalhoff³

et al. 1997

Eur Respir J

122

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“…GPA can be fatal if left untreated (Kaneishi et al 1995). An early diagnosis and treatment can prevent renal failure, which is the most common cause of death (Mark et al 1988). As trans-bronchial biopsies (TBLBs) of alveolar tissue are seldom positive in GPA with mild-to-moderate pulmonary involvement, unless they are taken from grossly abnormal lung areas, a surgical lung biopsy is necessary to confirm the diagnosis.…”

Section: Discussionmentioning

confidence: 99%

Pulmonary-Limited Granulomatosis with Polyangiitis Coexisting with Mixed Connective Tissue Disease

Ando

Goto

Yamasue

et al. 2017

Tohoku J. Exp. Med.

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Granulomatosis with polyangiitis (GPA) is a systemic disease characterized by necrotizing, granulomatous vasculitis of the upper and lower respiratory tracts and glomerulonephritis, and is classified as a classical or limited form. The classical form of GPA demonstrates the involvement of the upper respiratory tract, sinuses, lungs and kidneys, whereas the limited form is characterized by the lack of the renal involvement with female predominance. On the other hand, mixed connective tissue disease (MCTD) shows the clinical and laboratorial features of systemic lupus erythematosus, systemic sclerosis and polymyositis, along with high titers of anti-ribonucleoprotein antibodies and is characterized by good response to corticosteroid therapy and favorable prognosis. We herein report a patient with a history of MCTD that developed into a limited form of GPA (pulmonary-limited GPA). A 39-year-old woman suffered from persistent cough, left back pain and appetite loss. At 21 years of age she was diagnosed with MCTD, but the persistent administration of prednisolone or immunosuppressants was not needed. On admission, high-resolution chest computed tomography showed bilateral, multiple, poorly circumscribed nodules and masses, some of which showed cavitation. A surgical lung biopsy demonstrated granulomas with vasculitis surrounding the necrotic lesions. She was diagnosed with pulmonary-limited GPA. In conclusion, we should recognize that GPA may develop during the disease course of MCTD even after prolonged disease remission. To prevent progression to an irreversible state, physicians should consider a surgical lung biopsy for the diagnosis in patients suspected of having pulmonary-limited GPA.

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“…This was understandable, given the broad spectrum of "typical" and "atypical" histological lung injury patterns that may occur in WG or MPA (Table 2; [17][18][19][20][21][22][23][24][25][26][27][28][29][30]. Some of these "atypical" patterns (i.e., NSIP, BOOP, DAD, or pleuritis) overlapped with the findings that were seen in our patients, but no vasculitic changes were evident.…”

Section: Discussionmentioning

confidence: 99%

Antineutrophil Cytoplasmic Autoantibody in the Absence of Wegener's Granulomatosis or Microscopic Polyangiitis: Implications for the Surgical Pathologist

Gal

Velasquez

2002

Modern Pathology

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Antineutrophil cytoplasmic antibodies (ANCA) are useful serologic markers for the diagnosis and management of patients with Wegener's granulomatosis (WG) and microscopic polyangiitis (MPA). However, problems in diagnosis and classification may occur when patients with other disorders develop ANCA. A 7-year review (1993-1999) disclosed 247 patients whose sera tested positively for ANCA by an indirect immunofluorescence method: 166 patients for cytoplasmic-ANCA (C-ANCA) and 81 patients for perinuclear-ANCA (P-ANCA) Twenty-seven patients had active pulmonary disease and underwent openlung biopsy or transbronchial biopsy. Eight patients (30%) had a disease other than WG or MPA, and their clinical, pathological, and serological findings were reviewed. The patients, all women, ranged in age from 28 to 77 years (median, 37 y). Dyspnea (n ‫؍‬ 6), cough (n ‫؍‬ 6), chest pain (n ‫؍‬ 2), and/or hemoptysis (n ‫؍‬ 2) were present. The duration of symptoms lasted from 3 weeks to 6 years (median, 6 mo). ANCA titers were C-ANCA (n ‫؍‬ 4; range, 1:40 -1280) or P-ANCA (n ‫؍‬ 4; range, 1:40 -640). The lung biopsies disclosed nonspecific interstitial pneumonia (n ‫؍‬ 4), bronchiolitis obliterans organizing pneumonia (n ‫؍‬ 1), diffuse alveolar damage (n ‫؍‬ 1), organizing diffuse alveolar hemorrhage without capillaritis (n ‫؍‬ 1), and necrotic granuloma (n ‫؍‬ 1). No cases showed characteristic histology for WG or MPA. The final diagnoses were various connective tissue disorders (n ‫؍‬ 5), chronic hypersensitivity pneumonia (n ‫؍‬ 1), postinfectious bronchitis/bronchiectasis (n ‫؍‬ 1), and ulcerative colitis-related lung disease (n ‫؍‬ 1). Surgical pathologists should be aware that significantly elevated ANCA titers may be associated with diverse forms of pulmonary disease. ANCA positivity alone, in the absence of appropriate clinical or pathologic findings, should not be used to substantiate a diagnosis of WG or MPA.

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The pulmonary biopsy in the early diagnosis of Wegener's (pathergic) granulomatosis: A study based on 35 open lung biopsies

Cited by 142 publications

References 27 publications

Efficacy of transbronchial biopsy in pulmonary vaculitides

Efficacy of transbronchial biopsy in pulmonary vaculitides

Pulmonary-Limited Granulomatosis with Polyangiitis Coexisting with Mixed Connective Tissue Disease

Antineutrophil Cytoplasmic Autoantibody in the Absence of Wegener's Granulomatosis or Microscopic Polyangiitis: Implications for the Surgical Pathologist

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