Diagnosis and management of pulmonary vasculitis

Yunt, Zulma X.; Frankel, Stephen K.; Brown, Kevin K.

doi:10.1177/1753465812454693

Cited by 8 publications

(5 citation statements)

References 98 publications

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“…The clinical manifestations of WG are usually limited to the upper and/or lower respiratory tract although a number of other clinical features can arise . The lungs are commonly involved and this may range from asymptomatic nodules to pulmonary infiltrates and fulminant alveolar haemorrhage . Renal involvement most commonly consists of segmental necrotizing glomerulonephritis, though proliferative glomerulonephritis may also be observed .…”

Section: Introductionmentioning

confidence: 99%

Wegener's granulomatosis: a review of clinical features and an update in diagnosis and treatment

Almouhawis

Leão

Fedele

et al. 2013

J Oral Pathology Medicine

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Wegener's granulomatosis (WG) is an idiopathic, systemic inflammatory disease characterized by necrotizing granulomatous inflammation and pauci-immune small-vessel vasculitis of upper and lower respiratory tract and kidneys. The condition affects both genders equally, although some inconsistent gender differences have been observed. The aetiology of WG remains unknown although a number of exogenous factors have been suggested to be of aetiological relevance. Most clinical characteristics of this disease are non-specific, making clinical diagnosis challenging. Histopathological examination of lesional and peritoneal tissue is not pathognomonic, but is an essential investigation to confirm the presence of disease and exclude other disorders. At present, despite the increasingly wide range of potential therapies, cyclophosphamide plus corticosteroids remain the most recognized and effective means of inducing and sustaining remission of WG.

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Section: Introductionmentioning

confidence: 99%

Wegener's granulomatosis: a review of clinical features and an update in diagnosis and treatment

Almouhawis

Leão

Fedele

et al. 2013

J Oral Pathology Medicine

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“…The authors, however, note that induction therapy for GPA would typically include steroids in combination with either cyclophosphamide or methotrexate 5. In this case, remission was achieved quickly with steroid monotherapy, and azathioprine was then started as a maintenance agent.…”

Section: Discussionmentioning

confidence: 97%

A surprise behind a case of winter vomiting virus

2013

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A 79-year-old man was admitted through the medical take with norovirus gastritis. Routine plain chest radiography demonstrated a right coin lesion. CT and subsequent positron emission tomography showed a right upper lobe mass consistent with primary bronchial carcinoma. The lesion was resected and histology revealed a granulomatous necrotising mass without evidence of dysplasia. Meticulous investigations for infectious and non-infectious causes of necrotising granulomatous diseases were repeatedly negative. His postoperative recovery was complicated by a hospital-acquired pneumonia and a pulmonary embolism. CT pulmonary angiography showed progression of the previously resected mass and repeat biopsy was similar to the initial. A clinical diagnosis of antineutrophil cytoplasmic antibody-negative vasculitis without extrapulmonary manifestations was made and immunosuppressive therapy was initiated with rapid clinical response.

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“…Granulomatosis with polyangiitis (GPA, previously known as Wegener’s granulomatosis) is one of the ANCA-associated vasculitides (AAV), which involves the upper and lower respiratory tract and kidneys 2. It is a multisystem disorder characterised by necrotising granulomatous inflammation and pauci-immune vasculitis.…”

Section: Discussionmentioning

confidence: 99%

Granulomatosis with polyangiitis in a patient with biopsy-proven IgG4-related pulmonary disease and coincident small cell lung cancer

Abbass

Krug

2019

BMJ Case Rep

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Granulomatosis with polyangiitis (GPA) was diagnosed in a patient with a 16-month history of IgG4-related lung disease that spontaneously became asymptomatic. Cytoplasmic antineutrophil cytoplasmic antibody (ANCA) was positive at the time of diagnosis of IgG4-related disease (IgG4-RD), but there was no vasculitis or kidney disease. Sixteen months later he developed rapidly progressive glomerulonephritis that responded to cyclophosphamide treatment. While undergoing treatment for GPA, he was found to have a lung mass identified as small cell lung cancer. This mass was present at the time of the IgG4-RD diagnosis. GPA can be confused with IgG4-RD histologically and they rarely coexist. ANCA antibodies are primarily IgG4 subclass. IgG4-RD has been associated with cancer and may improve prognosis. We speculate that this patient may have had small cell lung cancer that incited an IgG4 predominant immune response with coexistent ANCA antibodies that eventually resulted in GPA. Immunosuppressive treatment of GPA likely accelerated the progression of the lung cancer.

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Diagnosis and management of pulmonary vasculitis

Cited by 8 publications

References 98 publications

Wegener's granulomatosis: a review of clinical features and an update in diagnosis and treatment

Wegener's granulomatosis: a review of clinical features and an update in diagnosis and treatment

A surprise behind a case of winter vomiting virus

Granulomatosis with polyangiitis in a patient with biopsy-proven IgG4-related pulmonary disease and coincident small cell lung cancer

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