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

doi:10.1378/chest.96.2.345

Chest

Volume 96, Issue 2, August 1989, Pages 345-347

https://doi.org/10.1378/chest.96.2.345 Get rights and content

Anti-neutrophil-cytoplasm antibodies recently have been reported as serologic markers of Wegener's granulomatosis. We describe two cases in which this test appeared to be of great value in the diagnosis of Wegener's granulomatosis presenting as acute respiratory failure, a clinical setting in which it may be the only diagnostic test that can be safely and easily performed. (Chest 1989; 96:345–47)

ANCA = anti-neutrophil-cytoplasm antibodies

Section snippets

CASE 1

A 69-year-old woman was admitted to the hospital with a one-month history of fever, productive cough with blood-streaked sputum, asthenia and anorexia. A few days before admission, she had conjunctivitis, which was treated with locally applied corticosteroids. For one year, she had complained of vertigo and headache. Physical examination showed a dullness and inspiratory crackles at the right lung base. Initial laboratory studies were as follows: WBC, 18,500/cu mm, with 70 percent neutrophils;

DISCUSSION

In 1985, Van der Woude et al⁴ reported the presence of IgG autoantibodies against cytoplasmic components of neutrophils (ANCA) in the serum of patients with Wegener's granulomatosis. Subsequently, ANCA were found in patients with classic polyarteritis nodosa, microscopic polyarteritis as well as in patients with necrotizing and crescentic glomerulonephritis.⁶^,⁷ Recently Falk and Jennette⁷ have reported positive reactions but with lower titers in a few patients with systemic lupus erythematosus

ACKNOWLEDGMENT

The ELISA was performed using a kit kindly provided by Dr. Wieslander (Biocarb, and University of Lund, Sweden).

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There are more references available in the full text version of this article.

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Alveolar Hemorrhage and Rare Infiltrative Diseases
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Prognostic factors for hospital mortality and ICU admission in patients with ANCA-related pulmonary vasculitis
2008, American Journal of the Medical Sciences
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.
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.
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.
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.
Respiratory failure in ANCA-associated vasculitis
1996, Chest
To assess the prevalence, clinical manifestations, and course of respiratory failure in all patients who tested positive for antineutrophil cytoplasmic autoantibodies (ANCA) in our clinics in the period between January 1985 and January 1993.
Case-series analysis.
Three teaching hospitals in the Netherlands.
Two hundred twenty consecutive patients suspected of having vasculitis and/or glomerulonephritis who tested positive for ANC A by indirect immunofluorescence and enzyme-linked immunosorbent assay.
Sixty-two patients had pulmonary involvement. Acute respiratory failure developed in nine. Respiratory failure was related to infections in two of them and to ANCA-associated vasculitis in seven. These seven patients uniformly presented with pulmonary hemorrhage and diffuse pulmonary infiltrates. The diagnosis of systemic vasculitis was supported by the presence of a pulmonary-renal syndrome in all patients, and by detection of antibodies to the proteinase 3 or myeloperoxidase antigen in all but one patient. Antiglomerular basement membrane antibodies were absent. The mortality was high due to hypoxic respiratory failure, pulmonary superinfections, and concomitant renal failure.
Acute respiratory failure due to vasculitis developed in one of every nine patients with ANCA-associated pulmonary disease. Patients usually present with pulmonary infiltrates and hemoptysis. A diagnosis of vasculitis may be further supported by analysis of the urinary sediment and determination of the ANCA target antigen. It remains to be proved that early detection of ANCA favorably affects the outcome.
Alveolar haemorrhage, glomerulonephritis and antineutrophil cytoplasmic antibodies
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L'intérêt des anticorps anticytoplasme de polynucléaires (ANCA) a été évalué dans le diagnostic et le suivi thérapeutique de 7 malades présentant une polyartérite microscopique ou une maladie de Wegener. Tous les malades avaient une glomérulonéphrite pauci-immune à nécrose segmentaire et focale, et une anémie. Cinq avaient une hémorragie alvéolaire avec hémoptysies et infiltrats radiographiques. Les ANCA, dosés par immunofluorescence indirecte sur neutrophiles fixés à l'éthanol étaient fortement positifs, avec un aspect cytoplasmatique pour 5 malades et périnucléaire pour 2. Une rémission initiale avec chute du titre d'ANCA était obtenue grâce aux corticoïdes, cyclophosphamide et parfois plasmaphérèse (5 malades). Des rechutes fréquentes avec réascension du titre d'ANCA survenaient lors de la diminution du traitement. En conclusion, les ANCA sont une aide précieuse au diagnostic des vascularites systémiques, en particulier lorsque l'hémorragie alvéolaire en est la forme inaugurale. Ils facilitent également la surveillance et les décisions thérapeutiques car les rechutes sont fréquentes.
The value of antineutrophilic cytoplasmic antibodies (ANCA) was assessed in the diagnosis and chronic treatment of 7 patients with microscopic polyarteritis or Wegener's granulomatosis. All patients had oligoimmune glomerulonephritis with segmental and focal necrosis and presented with anaemia. Five of them had alveolar haemorrhage with haemoptysis and infiltrates at radiography. ANCA were assayed by indirect immuno-fluorescence on ethanol-fixed neutrophils and were strongly positive, with a cycloplasmic aspect in 5 cases and a perinuclear aspect in 2 cases. Initial remission with fall in ANCA titres was obtained with corticosteroids, cyclophosphamide and sometimes plasmapheresis (5 patients), but frequent relapses with reelevation of ANCA titre occured when treatment was reduced. It is concluded that ANCA are very helpful in the diagnosis of systemic vasculitis, notably in cases with first-time alveolar haemorrhage. They also facilitate monitoring and therapeutic decisions, since relapses are frequent.
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: Manuscript received July 14; revision accepted December 9.

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Chest

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

Section snippets

CASE 1

DISCUSSION

ACKNOWLEDGMENT

Am J Med

Lancet

Lancet

Am J Kidney Dis

Wegener's granulomatosis: pathology and review of the literature

Arch Pathol