Vasculitis affecting the peripheral nerves predominantly manifests as subacute, progressive, asymmetric sensorimotor polyneuropathy or mononeuritis multiplex, and more rarely as painful mononeuropathy, pure sensory neuropathy, neuropathy of the cranial nerves, plexopathy, or as autonomic neuropathy. Vasculitic neuropathy may occur isolated or non-isolated (systemic) together with involvement of...

BACKGROUND Nasal deformity is a common disorder in Wegener granulomatosis, which is produced by polyangitis of medium-sized vessels. This process may cause necrosis and destruction of the nasal mucosa and osteocartilaginous framework. At this time, there is a lack of published data on nasal deformities associated with Wegener granulomatosis. METHODS We present a 53-year-old woman with complet...

INTRODUCTION Granulomatosis with polyangitis (Wegener's) is an antineutrophil cytoplasmic antibody (PR3-ANCA)-associated vasculitis, which commonly involves the upper and lower respiratory tracts and kidneys. Central nervous system involvement is reported in less than 11%, and rarely present at onset. CASE OUTLINE We report the case of a 41-year-old male patient with a high disease activity, ...

BACKGROUND Pulmonary fibrosis is a manifestation of microscopic polyangitis (MPA), and often precedes the detection of MPA. The prevalence and sequence of myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA) and MPA in patients initially diagnosed with idiopathic pulmonary fibrosis (IPF) have not been precisely elucidated. METHODS We enrolled 61 consecutive patients with IPF and me...

Eosinophilic granulomatosis with polyangitis (EGPA) is an uncommon ANCA-associated systemic small-vessel necrotizing vasculitis. At times, EGPA presenting manifestations can be very different from the usually recognized disease patterns. We report a 52-year-old female patient with 3 years history of itching. During the time occurred a chronic skin lichenification on her legs and gradually devel...

To determine the patterns and outcomes of the pauci-immune vasculitis in the nephrology department at hospital La Conception in Marseille, we conducted a retrospective study including all patients with diagnosis of pauci-immune renal vasculitis between January 1, 2000 and December 31, 2007. Among 33 cases, 25 were diagnosed as Wegener granulomatosis (WG), seven as microscopic polyangitis (MPA) ...