Necrotizing, small-vessel vasculitis develops in patients with circulating ANCAs. Neutrophils and monocytes harbor the two major ANCA antigens, PR3 and MPO. A whole body of in vitro experiments implicated ANCA-activated neutrophil effector functions in the pathogenesis of vasculitis, whereas the role of monocytes is less well-characterized. Mouse models for anti-MPO-induced vasculitis were deve...

Prolastin is a commercially available form of alpha-1-antitrypsin (AAT) that is derived from pooled human plasma and used for treatment of severe alpha-1-antitrypsin deficiency (AATD). We describe a patient with AATD who developed presumed hypersensitivity vasculitis (HV) following a Prolastin infusion. Hypersensitivity vasculitis (HV), or cutaneous vasculitis, is characterized by inflammation ...

Cutaneous small-vessel vasculitis (CSVV) is a general term for diseases that present pathological features of leukocytoclastic vasculitis, i.e., diseases with pathological perivascular neutrophilic infiltration and fibrinoid degeneration of the vascular walls (Fig. 11.1). Henoch-Schönlein purpura and urticarial vasculitis are included under a broad definition of CSVV; however, they are usually ...

OBJECTIVE Assessment of disease activity in vasculitis can be achieved using the BVAS, a clinical checklist of relevant symptoms, signs and features of active disease. The aim of this study was to revalidate the BVAS version 3 (BVAS v. 3) in a cohort of patients with systemic vasculitis. METHODS A total of 238 patients with vasculitis from seven countries in Europe were evaluated at a single ...

PURPOSE OF REVIEW In the past decade, primary and secondary central nervous system (CNS) vasculitides have been more commonly diagnosed and recognized than previously. With the increasing awareness of these disorders, it is crucial for the treating physician to differentiate between causes of CNS vasculitis and to recognize their marked clinical and pathophysiological heterogeneity. This review...

Systemic vasculitis involving the lung is a rare manifestation of myelodysplastic syndrome (MDS), and secondary vasculitis is considered to have poor prognosis. A 44-year-old man presented with fever and dyspnea of 1 month duration. A chest radiograph revealed bilateral multiple wedge shaped consolidations. In addition, the results of a percutaneous needle biopsy for non-resolving pneumonia wer...

The occurrence of perinuclear anti-neutrophilic cytoplasmic antibodies (p-ANCA) in pulmonary fibrosis has been reported in a number of small studies, however, the appearance of p-ANCA in already established idiopathic pulmonary fibrosis (IPF), predating the manifestations of vasculitis has not been widely appreciated. We report 2 cases with radiographic evidence of established IPF where p-ANCA ...

OBJECTIVE To describe vasculitis in HIV patients, their CD4 levels, anatomical sites affected and clinical patterns. DESIGN Prospective, descriptive study. SETTING Kisumu District Hospital and Nairobi Rheumatology Clinic between January 2002 and May 2005. SUBJECTS Eight patients with HIV and vasculitis. RESULTS Eight patients (four males and four females) were recruited with an age rang...

OBJECTIVES ANCA-associated vasculitis and interstitial lung disease (ILD) are uncommon conditions. The occurrence of both diseases in the same patient is increasingly recognized. Our aim was to ascertain the characteristics and outcomes of patients with ILD and ANCA-associated vasculitis. METHODS A retrospective observational cohort study was performed. Patients who presented to the Hammersmi...

BACKGROUND Pulmonary haemorrhage (PH) is a serious manifestation of systemic vasculitis with high mortality rates yet vasculitis is associated with an increased prevalence of venous thromboembolism (VTE). The concurrent presentation of severe PH and VTE poses a challenge in terms of therapeutic management. METHODS This is a retrospective case review of the clinical manifestations and response...