Wegener's Granulomatosis Presenting as Acute Respiratory Failure with Anti-neutrophil-cytoplasm Antibodies

Lenclud, Christine; Vuyst, Paul De; Dupont, Étienne; Depierreux, Michel; Ketelbant, P.; Goldman, Michel

doi:10.1378/chest.96.2.345

Cited by 20 publications

(8 citation statements)

References 9 publications

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“…Furthermore, otitis media occurs in 18to 25% of patients with Wegener'sgranulomatosis (18,19), and upper airway symptoms in general occur in 73% at diagnosis (20). However, cases of active Wegener'sgranulomatosis are ANCA-positive (21), and more specifically, in cases associated with capillaritis and DAH, patients have been ANCApositive whether disease was limited to the respiratory tract or not (5)(6)(7). All ANCA measurements in our subjects were performed before lung biopsy and treatment.…”

Section: Discussionmentioning

confidence: 99%

“…However, c-ANCA is positive, and the more typical clinical and histologic findings of Wegener's granulomatosis appear at a later date (5,6). When pulmonary capillaritis and DAH occurs in association with microscopic polyangiitis (the small-vessel variant of polyarteritis nodosa) or systemic lupus erythematosus, respiratory manifestations are also accompanied by multisystem disease, particularly glomerulonephritis (7,8).…”

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confidence: 99%

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Diffuse alveolar hemorrhage with underlying isolated, pauciimmune pulmonary capillaritis.

Jennings¹,

King²,

Tuder³

et al. 1997

Am J Respir Crit Care Med

117

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Diffuse alveolar hemorrhage (DAH) resulting from pulmonary capillaritis typically accompanies the systemic vasculitides and collagen vascular diseases. Isolated pulmonary capillaritis and DAH without systemic disease occurs in patients with antineutrophil cytoplasmic antibodies. However, isolated pulmonary capillaritis and DAH is not described for patients without clinical or serologic evidence for an underlying systemic disease. To describe such patients, we undertook a retrospective chart review of 29 patients with DAH and biopsy-proven pulmonary capillaritis from seven Denver hospitals. Eight (28%) were diagnosed with isolated pulmonary capillaritis without clinical, serologic, or histologic evidence of an associated illness. Their median age was 30 yr. No association with occupational or drug exposures was identified. All had lower respiratory tract symptoms; seven had upper respiratory tract symptoms. None demonstrated systemic disease or evidence of glomerulonephritis. All were antineutrophil cytoplasmic antibody negative. Other serologies were not significant where measured. Direct immunofluorescence studies of lung tissue were negative in five. Six presented with acute respiratory failure, four requiring mechanical ventilation. One died during initial hospitalization; seven survived. Median follow-up is 43 mo (7 to 73 mo). Five remain in remission. Two experienced multiple recurrences of DAH but without development of systemic disease while on therapy. Herein we characterize DAH and isolated pulmonary capillaritis in the absence of clinical, serologic, or histologic evidence indicating an accompanying systemic illness. The prognosis for this group appears favorable.

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Section: Discussionmentioning

confidence: 99%

mentioning

confidence: 99%

Diffuse alveolar hemorrhage with underlying isolated, pauciimmune pulmonary capillaritis.

Jennings¹,

King²,

Tuder³

et al. 1997

Am J Respir Crit Care Med

117

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“…Subsequent research showed an association between the presence of these antibodies and Wegener’s granulomatosis, Churg-Strauss, and microscopic polyangiitis. 2–6 Clinical manifestations of ANCA-associated vasculitis include a progressive type of glomerulonephritis and/or pulmonary complications such as capillaritis and diffuse alveolar hemorrhage. Pulmonary capillaritis has been reported in 12% to 29% of patients with microscopic polyangiitis and in 17% to 35% of patients with Wegener’s granulomatosis.…”

mentioning

confidence: 99%

Prognostic Factors for Hospital Mortality and ICU Admission in Patients With ANCA-Related Pulmonary Vasculitis

Holguín¹,

Ramadan²,

Gal

et al. 2008

The American Journal of the Medical Sciences

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Background The objective of this study was to evaluate the factors predictive of 28-day mortality and admission to Intensive Care Unit (ICU) in patients with ANCA-related pulmonary vasculitis. Methods We reviewed the medical records and imaging studies of 65 patients diagnosed with ANCA-related vasculitis hospitalized with pulmonary complications between February 1985 and November 2002. All patients underwent open or video-assisted thoracoscopic lung biopsy, had a positive ANCA serology, and were negative for glomerular basement membrane antibodies. Results At presentation, 72% had dyspnea, 68% fever, 47% cough, 45% elevated blood pressure, 32.3% hemoptysis, 26.1% sinus involvement, 15% renal failure, and 4.6% scleritis. Pathological findings included alveolar hemorrhage (60%), granulomatous inflammation (46%), and capillaritis (38%). A significant number required mechanical ventilation (27.7%), hemodialysis (24.6%), continuous renal replacement therapy (3.1%), and plasmapheresis (3.1%). The 28-day mortality was 16.9% (11/65). Mechanical ventilation (OR 68, P < 0.005), admission to ICU (OR 18.5, P < 0.01), and blood transfusion (OR 22.4, P < 0.004) were strong predictors of increased mortality within 28 days after admission. Respiratory failure (OR 31, P < 0.0007), hemoptysis (OR 2.9, P < 0.06), smoking (OR 5.9, P < 0.02), and acute renal failure (OR 7.8, P < 0.01) were also predictors for admission to the ICU. Conclusion In patients with ANCA-related pulmonary vasculitis several clinical factors, but not pathologic findings or ANCA titers, are associated with ICU admission and/or 28-day mortality.

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“…Other cases of granulomatous vasculitis associated with DAH have been discerned. [59][60][61][62][63] DAH developed in two children with WG. 64,65 Conversely, capillaritis was seen in a series of all 36 patients with ANCAs and DAH, but granulomatous inflammation was not present in any case, including eight with WG.…”

Section: Microscopic Polyangiitismentioning

confidence: 99%

Alveolar Hemorrhage in Vasculitis: Primary and Secondary

Cordier

Cottin

2011

Semin Respir Crit Care Med

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Diffuse alveolar hemorrhage (DAH) in primary and secondary vasculitis occurs when capillaritis is present. The diagnosis of DAH is considered in patients who develop progressive dyspnea with alveolar opacities on chest imaging (with density ranging from ground glass to consolidation) that cannot be explained otherwise. Hemoptysis, a valuable sign, is often absent. A decline of blood hemoglobin level over a few days without hemolysis or any hemorrhage elsewhere should be an alert for DAH. Bronchoalveolar lavage, retrieving bright red fluid, is the best diagnostic clue. Lung biopsy is not recommended. A search for anti-neutrophil cytoplasmic autoantibodies (ANCAs) is mandatory. Once DAH is diagnosed and hemodynamic as well as infectious causes have been excluded, ANCA-associated vasculitis is taken into account (mainly microscopic polyangiitis or Wegener granulomatosis, and, exceptionally, Churg-Strauss syndrome). Drug-induced DAH, especially antithyroid drugs such as propylthiouracil may be coupled with ANCA. Isolated DAH with capillaritis with or without ANCA is rare. DAH in systemic lupus erythematosus is either associated or not with capillaritis. Treatment of DAH should target the underlying disorder. In the primary vasculitides, corticosteroids and immunosuppressants, especially cyclophosphamide, are the mainstay of therapy, but plasma exchange, particularly in severe DAH, is the rule, although evidence of its effectiveness is awaited.

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Wegener's Granulomatosis Presenting as Acute Respiratory Failure with Anti-neutrophil-cytoplasm Antibodies

Cited by 20 publications

References 9 publications

Diffuse alveolar hemorrhage with underlying isolated, pauciimmune pulmonary capillaritis.

Diffuse alveolar hemorrhage with underlying isolated, pauciimmune pulmonary capillaritis.

Prognostic Factors for Hospital Mortality and ICU Admission in Patients With ANCA-Related Pulmonary Vasculitis

Alveolar Hemorrhage in Vasculitis: Primary and Secondary

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