The Pulmonary Vasculitides

Division of Pulmonary Sciences and Critical Care Medicine, Department of Medicine, Anschutz Medical Campus, University of Colorado, Aurora, Colorado, USA.
American Journal of Respiratory and Critical Care Medicine (Impact Factor: 13). 06/2012; 186(3):216-24. DOI: 10.1164/rccm.201203-0539CI
Source: PubMed


The pulmonary vasculitides are a rare group of heterogeneous disorders unified by the histopathologic finding of inflammation and destruction of the blood vessel wall. Diagnosis of these disorders is exceptionally challenging, given their highly variable clinical presentation, their relative rarity, and the overlap of the signs and symptoms of vasculitis with much more common entities. However, advances in the management of vasculitis allow for accurate diagnosis, risk stratification in the individual patient, and the implementation of evidence-based, effective pharmacologic therapies. This concise clinical review addresses the diagnosis and management of the patient with pulmonary vasculitis and provides an up-to-date review of the state of the field.

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    • "In patients with GPA, conventional induction therapy usually consists of high dose glucocorticoids and cyclophosphamide [5]. When administered shortly after the induction therapy, combined immunosuppressive therapy ensures remission in up to 90% of patients within 6 months [12]. "
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    ABSTRACT: Granulomatosis with polyangiitis (GPA) is a rare form of vasculitis. Multidisciplinary therapeutic approach and early diagnosis assume vital importance in management of patients with diffuse alveolar haemorrhage caused by GPA, which is a rare complication. The purpose of this study was to present the diagnostic and therapeutic challenges experienced by clinicians in management of two severe cases of GPA with insidious extrapulmonary manifestations which rapidly progressed into acute kidney injury, alveolar haemorrhage and acute respiratory failure.
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    • "Subjects’ demographics, initial symptoms, date of diagnosis, physical examination findings, ANCA levels, blood biochemistry, diagnostic imaging, relapses and treatment regimens were recorded. Relapses are defined within the newly developed clinical finding of GPA and adapted to the diseases according to BVAS definition [6]. Cytoplasmic or proteinase 3(c-/PR3) and perinuclear or myeloperoxidase (p-/MPO) ANCA were measured at the same time point using ELISA. "
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    ABSTRACT: Background Antineutrophilic cytoplasmic antibody (ANCA)-associated vasculitis (AAV) may present with pulmonary involvement ranging from mild to life-threatening disease such as diffuse alveolar hemorrhage. There is a paucity of information regarding morbidity outcomes for AAV subjects presenting with lung involvement. This study determines the relationship between disease activity and damage in these subjects using the Birmingham Vasculitis Activity Score v 3 (BVAS 3) and Vasculitis Damage Index (VDI) respectively. Results 151 patients with AAV were included with 59 presenting initially with pulmonary involvement. The initial BVAS scores recorded at time of diagnosis were positively correlated with the final VDI scores at 24 months (p < 0.0001, rs = 0.5871). No differences between BVAS and VDI scores were seen for both groups, however in the lung-involvement group only, BVAS scores were significantly higher at 6, 12 and 24 months whilst the VDI scores were significantly higher at 12 and 24 months. Subjects presenting with pulmonary involvement had an increased likelihood for cardiovascular (OR 1.31, 95% CI 0.89, 1.54; p = 0.032) and renal (OR 1.32, 95% CI 1.22, 1.39; p = 0.005) involvement. Subjects presenting with lung involvement with granulomatosis with polyangiitis and microscopic polyangiitis had 24-month VDI scores that were significantly higher (p = 0.027, p = 0.045), and more likely to develop pulmonary fibrosis (OR 1.79, 95% CI 1.48, 2.12; p < 0.001). Conclusion AAV subjects with lung involvement at presentation had a higher disease activity and damage scores at 6, 12 and 24 months follow-up representing a considerable burden of disease despite improvement in overall survival due to the introduction of immunosuppressive therapy.
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    • "Although severity and prognosis are dependent on a number of factors, the most important of which seems to be the severity of disease activity as measured by the number of organ systems involved, the degree of renal disease, and the presence of DAH. Based on these associations, the European Vasculitis Study Group (EUVAS) has devised a clinically useful grading system (Table 2) in which the patient's disease is categorized as 1) limited, 2) early, generalized, 3) active, generalized, 4) severe, or 5) refractory13,14. "
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    ABSTRACT: Diffuse alveolar hemorrhage (DAH) is a life-threatening and medical emergency that can be caused by numerous disorders and presents with hemoptysis, anemia, and diffuse alveolar infiltrates. Early bronchoscopy with bronchoalveolar lavage is usually required to confirm the diagnosis and rule out infection. Most cases of DAH are caused by capillaritis associated with systemic autoimmune diseases such as anti-neutrophil cytoplasmic antibody-associated vasculitis, anti-glomerular basement membrane disease, and systemic lupus erythematosus, but DAH may also result from coagulation disorders, drugs, inhaled toxins, or transplantation. The diagnosis of DAH relies on clinical suspicion combined with laboratory, radiologic, and pathologic findings. Early recognition is crucial, because prompt diagnosis and treatment is necessary for survival. Corticosteroids and immunosuppressive agents remain the gold standard. In patients with DAH, biopsy of involved sites can help to identify the cause and to direct therapy. This article aims to provide a general review of the causes and clinical presentation of DAH and to recommend a diagnostic approach and a management plan for the most common causes.
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