Diagnosis and classification of granulomatosis with polyangiitis (aka Wegener's granulomatosis)

Lutalo, Pamela; D’Cruz, David

doi:10.1016/j.jaut.2014.01.028

Cited by 205 publications

(197 citation statements)

References 36 publications

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“…GPA has an incidence of 5-10 cases per million population with males and females equally affected. [1] According to American College of Rheumatology (ACR, 1990), GPA is defined by the presence of at least two of the four criteria: 1) nasal or oral inflammation, 2) abnormal chest radiograph with either the presence of nodules, fixed infiltrates or cavities, 3) urine sediment with hematuria or red cell cast, and 4) granulomatous inflammation on biopsy within an artery or in the perivascular area of an artery or arteriole. [3] Our patient fulfilled the ACR criteria by having the evidence of pulmonary lesions and granulomatous inflammation seen in the skin, gum and kidney biopsies.…”

Section: Discussionmentioning

confidence: 99%

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A case of fatal gastrointestinal hemorrhage in granulomatosis with polyangiitis

Wang

Chua

Ranasinghe

et al. 2017

CRIM

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Although granulomatosis with polyangiitis (GPA) can affect any organ, gastrointestinal (GI) involvement is uncommon. Moreover, death from massive GI hemorrhage is a rare entity, with few cases described in the literature. In our report, we present a case of a patient with GPA who developed massive ulcerative bleeding, which ultimately proved fatal. Given the paucity of reports available, the significant potential for morbidity, and the fact that concurrent immunosuppressive therapy may themselves exacerbate the risk of bleeding, we reviewed 49 case reports of patients with GPA and GI involvement from 1982 to June 2016 in an attempt to shed light on a little seen sequelae that warrants a high index of suspicion.

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Section: Discussionmentioning

confidence: 99%

“…[1] Gastronintestinal (GI) manifestations in GPA are uncommon, but the morbidity from GI involvement can be severe. [2] In our case report, we describe an unusual case of GPA that presented with catastrophic upper GI ulcers that proved fatal.…”

Section: Introductionmentioning

confidence: 99%

A case of fatal gastrointestinal hemorrhage in granulomatosis with polyangiitis

Wang

Chua

Ranasinghe

et al. 2017

CRIM

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“…Both of these entities may involve the sinonasal tract, although GPA more commonly does. 2,3,[25][26][27][28] Granulomatous polyangiitis is a necrotizing vasculitic process associated with cytoplasmic antinuclear cytoplasmic antibodies (c-ANCA). It is a systemic disease that is most commonly associated with necrotizing granulomatous vasculitis of the upper and lower respiratory tract and kidneys.…”

Section: Autoimmune Disease/vasculitis: Antineutrophilic Cytoplasmic mentioning

confidence: 99%

Differential Diagnosis of Necrotizing Sinonasal Lesions

Montone

2015

Archives of Pathology &Amp; Laboratory Medicine

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Context A number of entities may result in necrosis in the sinonasal tract and lead to significant morbidity and mortality. These include infections, necrotizing vasculitis, neoplastic processes, and drug dependency. This review will concentrate on the differential diagnosis of sinonasal necrotizing lesions. Objective To review the differential diagnoses of necrotizing destructive lesions of the sinonasal tract. Data Sources The current literature was reviewed to provide updated information regarding the differential diagnosis of sinonasal necrotizing lesions, including infectious disease processes; antineutrophilic cytoplasmic antibody–associated vasculitides; neoplastic processes, particularly natural killer/T-cell lymphomas; and drug abuse. Conclusions The differential diagnosis of necrotizing sinonasal lesions is broad, with often overlapping diagnostic features that lead to diagnostic challenges. Ancillary tests such as special stains and immunohistochemical studies can offer significant assistance.

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“…4 Choosing the type of treatment depends on the affected organ and the severity of the disease. 5 Treatment is usually initiated with glucocorticoids such as prednisolone and cytotoxic drugs such as cyclophosphamide, methotrexate, or azathioprine. 5 This rare syndrome has an incidence rate of about 3 in 100 000 with a higher occurrence rate in the fourth and fifth decades of life in both men and women.…”

Section: Introductionmentioning

confidence: 99%

“…5 Treatment is usually initiated with glucocorticoids such as prednisolone and cytotoxic drugs such as cyclophosphamide, methotrexate, or azathioprine. 5 This rare syndrome has an incidence rate of about 3 in 100 000 with a higher occurrence rate in the fourth and fifth decades of life in both men and women. 6 In the case presented here, however, the disease occurred in a younger subject and had an unusual progression.…”

Section: Introductionmentioning

confidence: 99%