Classification of ANCA-associated vasculitis: differences based on ANCA specificity and clinicopathologic phenotype.

The classification of vasculitis according to a schema with universal acceptance is challenging, given the heterogeneous and protean nature of these diseases. Formal nomenclature and classification criteria for anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) have suffered several changes since their first description; none provides comprehensive diagnostic and classification criteria. Different factors account for the difficulties in the classification of vasculitis, including the incomplete understanding of the pathogenesis, the multisystemic nature of the disease, the non-specific patterns of vascular involvement, the overlap between entities, and the presence of various classification systems. The present article reviews the classification of AAV considering different points of view, including clinical, serologic, pathogenetic, organ predilection, therapeutic, and prognostic factors, and provides perspectives on future challenges in the understanding of AAV. There is an unmet need for a unifying view of the disease spectrum that considers the constantly evolving paradigms.

Pulmonary Involvement in Systemic Vasculitis.

PURPOSE OF REVIEW: The purpose of this study is to describe the most relevant advances concerning lung involvement in the ANCA-associated vasculitides (excluding eosinophilic granulomatosis with polyangiitis which may have different disease mechanisms). Focus is on pathophysiology, recent important imagenological procedures, treatment, and outcome. RECENT FINDINGS: Emerging information exists on potential newly investigated diagnostic procedures (v.g. transbronchial cryobiopsies), detailed tomographic abnormalities, the potential favorable role of rituximab and the still uncertain one of plasma exchange in the treatment, and the increasing description of interstitial lung disease. Survival is reduced in case of both, diffuse alveolar hemorrhage and diffuse parenchymal disease. There is the need to expand the knowledge concerning better long-term treatment options with specific regimes, and to incorporate other measures regarding integral treatment in patients afflicted with lung involvement these maladies, as the outcome seems adverse in this scenario.

[Clinical Characteristics of antineutrophil cytoplasmic antibody-associated vasculitis in a respiratory diseases referral center in Mexico (1982-2010)]. / Vasculitis asociadas a anticuerpos anticitoplasma de neutrófilos (ANCA) en un centro de tercer nivel de enfermedad respiratoria (1982-2010).

INTRODUCTION: Respiratory manifestations in antineutrophil cytoplasmic antibody-associated vasculitis (AASV) are common, though their suspicion is lower than expected in respiratory devoted centers, with few descriptions coming from them. OBJECTIVE: To describe the clinical, paraclinical and radiological manifestations, plus the prognosis of AASV patients seen in a respiratory referral center in Mexico City. MATERIAL AND METHODS: Retrospective review of patients with final diagnosis of AASV, based on the American College of Rheumatology criteria and the 1994 Chapel Hill Consensus Conference Nomenclature, from 1982 to 2010. RESULTS: The characteristics of 74 granulomatosis with polyangiitis, 10 microscopic polyangiitis, and six eosinophilic granulomatosis with polyangiitis cases are described. Mean time elapsed from initial suspicion to definitive diagnosis was 30 months. As expected, respiratory findings dominated this cohort, but no significant differences were observed when compared to other series with AAS\1, except for a higher frequency of subglottic stenosis. After a mean follow-up of 22 months, 83% of patients were alive, with remission being achieved in 87% and response in 9%. Seven patients died, mostly from infectious complications. CONCLUSION: This study documents that airway manifestations in Mexican patients with AASV are similar to what has been previously described. However, time to diagnosis is long. Respiratory specialists should be more aware of the modes of presentation in AASV patients in order to facilitate their recognition.

Giant cell arteritis in Mexican patients.

BACKGROUND: Giant cell arteritis (GCA) is the most common primary systemic vasculitis worldwide, although it seems to be very rare in some areas, such as Latin America. OBJECTIVES: The objective of the study was to describe the clinical, laboratory, and treatment features in a Mexican Mestizo population with GCA. METHODS: Retrospective data chart review (1989-2010). RESULTS: Twenty-two patients with GCA were identified, 18 women and 4 men. Mean age was 73 (SD, 7.9) years. Diagnosis was made at a mean of 67 (SD, 83.6) days from symptom onset. Most frequent presenting symptoms included headache (90%), constitutional symptoms (86%), and polymyalgia rheumatica (59%). Severe cranial ischemic complications were present in 32%. Amaurosis fugax and blindness were present in 36% and 27%, respectively. High erythrocyte sedimentation rate was present in 89% of patients. Rapid response to prednisone treatment was seen, but in 10 patients, relapse occurred, possibly related to fast tapering. Additional treatment was methotrexate (n = 8), azathioprine (n = 5), and cyclophosphamide (n = 3). Median follow-up was 242 (SD, 214) weeks. CONCLUSIONS: Giant cell arteritis is rarely recognized in Latin America. We report on characteristics of GCA in a population of Mexican Mestizos, as ours is the largest series to be reported from Latin America so far. When compared with other series, age at onset is similar, females are more affected, and although a good response to corticosteroid treatment was seen, a higher frequency of amaurosis fugax and blindness was observed, accounting for an unfavorable functional outcome in 6 (27%) of 22 patients.