Pulmonary manifestations of anti-neutrophil cytoplasmic antibody (ANCA)-positive vasculitis

Thickett, David; Richter, Alex; Nathani, Nazim; Perkins, Gavin D.; Harper, Lorraine

doi:10.1093/rheumatology/kei217

Cited by 63 publications

(45 citation statements)

References 83 publications

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“…All 3 patients' flow-volume curve indicated an obstructive ventilatory defect as reported by a previous study [17], even though 2 of them had no smoking history. Pathological findings which showed granulomatous bronchiolitis explained this result.…”

Section: Discussionsupporting

confidence: 66%

“…In our previous study with MPO-ANCA-positive UIP, we showed the importance of surgical lung biopsy to diagnose IP associated with MPO-ANCA [18]. In our present cases, the pathological examination demonstrated vasculitis with granuloma and microabscess which were compatible with GPA [17], although the radiological finding was not typical for GPA. Especially in case 3, even the kidney biopsy did not lead to the diagnosis of GPA because of the lack of necrotizing crescentic glomerulonephritis.…”

Section: Discussionsupporting

confidence: 50%

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Myeloperoxidase Anti-Neutrophil Cytoplasmic Antibody-Positive Interstitial Pneumonia Associated with Granulomatosis with Polyangiitis Diagnosed by Surgical Lung Biopsy

et al. 2016

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Granulomatosis with polyangiitis (GPA) is a rare systemic vasculitis that often involves the lung. However, interstitial pneumonia (IP) is rarely seen in GPA patients. We herein report 3 cases of IP associated with GPA diagnosed by surgical lung biopsy. High-resolution CT showed uniform subpleural reticular opacity with traction bronchiectasis. Biopsies from all 3 patients revealed neutrophilic capillaritis, microabscesses with giant cells, and coexisting histological findings of usual IP pattern or fibrosing nonspecific IP pattern. All 3 patients had elevated levels of serum myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA), but not proteinase 3-ANCA. We diagnosed GPA and treated with corticosteroid and cyclophosphamide. Follow-up CT showed improvement of the lesions in all patients. Surgical lung biopsy specimens which revealed GPA enabled us to conduct the most suitable therapy. This report indicates the importance of surgical lung biopsy for differentiating idiopathic IPs from GPA-associated IP and suggests a relationship between MPO-ANCA and IP in GPA.

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Section: Discussionsupporting

confidence: 66%

Section: Discussionsupporting

confidence: 50%

Myeloperoxidase Anti-Neutrophil Cytoplasmic Antibody-Positive Interstitial Pneumonia Associated with Granulomatosis with Polyangiitis Diagnosed by Surgical Lung Biopsy

et al. 2016

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“…1-3). In addition, pleural effusion, pleural irregularities, hilar and mediastinal lymphadenopathy, and tracheal wall abnormalities are present equally in about 15% of cases [7]. Although uncommon, lymphadenopathy when present usually involves the right paratracheal and hilar nodes.…”

Section: Granulomatosis With Polyangiitismentioning

confidence: 99%

The Respiratory System in Autoimmune Vascular Diseases

Nasser¹,

Cottin²

2018

Respiration

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The respiratory system may be involved in all types of systemic vasculitis with varying significance and frequency. ANCA-associated vasculitis, including granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, and microscopic polyangiitis, affects the respiratory tract more commonly than other vasculitis types. Granulomatosis with polyangiitis is always associated with upper or lower respiratory tract involvement. Pulmonary and ENT involvements are the hallmark feature of the disease and are present in 90 and 80% of cases, respectively, with frequent skin or gastrointestinal involvement. In about 10% of cases, the lung is the only organ affected. Eosinophilic granulomatosis with polyangiitis is always associated with hypereosinophilia and asthma which usually precedes the systemic manifestations by several years; however, onset of asthma and of the vasculitis may be concomitant. Parenchymal infiltrates may be migratory and rapidly resolve upon corticosteroid treatment. Diffuse alveolar hemorrhage and renal failure are typical features of microscopic polyangiitis. The former is the leading manifestation of anti-glomerular basement membrane disease and is usually part of a pulmonary-renal syndrome. Takayasu arteritis has a distinct clinical presentation due to pulmonary arteritis and may present with massive hemoptysis, chest pain, and rarely symptoms of pulmonary hypertension. Behçet disease is the most common cause of pulmonary artery aneurysm and can also cause in situ thrombosis of the pulmonary arteries. Corticosteroids and immunosuppressive agents are the mainstay of treatment. In conclusion, systemic vasculitis is a frequent cause of respiratory system involvement with diverse manifestations of distinct severity and outcome.

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“…3 Radiological presentation may be in the form of interstitial, mixed, or alveolar infi ltrates, or nodular or cavitary disease. 10,11,14,15 The most common radiographic fi ndings include pulmonary infi ltrate (67%) and nodules (58%). 12 Nodules are predominantly subpleural and are cavitated in nearly 50% of cases.…”

Section: Discussionmentioning

confidence: 99%

Limited Wegener's Granulomatosis as a Solitary Pulmonary Nodule - A Case Report

Mishra

Singla

Dewan

2013

SAARC J. Tuber. Lung Dis. HIV/AIDS

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Limited Wegener`s Granulomatosis (WG) has been infrequently reported from India so far. We report a case of Limited WG in a 60 year old male, who presented to us with productive cough and hemoptysis, and a right upper lobe mass lesion on chest roentgenogram. He was previously treated outside as Pulmonary Koch's, without any improvement. We diagnosed the patient on histo-pathological examination of right upper lobectomy specimen. He was administered fi ve pulses of intravenous cyclophosphamide therapy plus oral corticosteroids, with signifi cant symptomatic improvement. He was given oral methotrexate and tapering doses of corticosteroids in follow up.

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Pulmonary manifestations of anti-neutrophil cytoplasmic antibody (ANCA)-positive vasculitis

Cited by 63 publications

References 83 publications

Myeloperoxidase Anti-Neutrophil Cytoplasmic Antibody-Positive Interstitial Pneumonia Associated with Granulomatosis with Polyangiitis Diagnosed by Surgical Lung Biopsy

Myeloperoxidase Anti-Neutrophil Cytoplasmic Antibody-Positive Interstitial Pneumonia Associated with Granulomatosis with Polyangiitis Diagnosed by Surgical Lung Biopsy

The Respiratory System in Autoimmune Vascular Diseases

Limited Wegener's Granulomatosis as a Solitary Pulmonary Nodule - A Case Report

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