A retrospective study of 48 patients was conducted to evaluate the efficacy of plasma exchange in children with idiopathic rapidly progressive glomerulonephritis (IRPGN), and renal or non-renal vasculitis. All patients were followed up at a single centre over a 15 year period. Treatment consisted of corticosteroids and/or cytotoxic agents. Plasma exchange was used in all patients because of sev...

This special edition reviews the progress in understanding of systemic vasculitis associated with autoantibodies to neutrophil cytoplasmic antigens (ANCA). European research groups have contributed to this research including the original observations of the association between ANCA and vasculitis. Areas of interest include classification and epidemiology, genetics and pathogenesis, disease asse...

OBJECTIVES To establish the clinical significance of isolated nail fold vasculitis in patients with rheumatoid arthritis (RA). METHODS Patients attending hospital with established RA were assessed by a single observer using unaided vision. Isolated nail fold vasculitis (NFV) was diagnosed as small nail edge or nail fold lesions without any evidence of other extra-articular or systemic vasculi...

Primary systemic vasculitides in children are relatively rare diseases. In most cases, they have an unknown etiology and are defined as the presence of inflammation in the blood vessel wall. Establishing the diagnosis of vasculitis is oft en challenging, since the disorder is multisystem in nature with mostly nonspecific symptoms. The last 10 years have seen significant advances in the field of...

Several vasculitic syndromes are recognized as paraneoplastic syndromes of an underlying malignant disease. Most frequently small vessel vasculitis of the skin has been reported. We describe the case of a 62-year-old man with a pulmonary mass due to pulmonary vasculitis. After resection of the pulmonary mass, the patient displayed bone metastasis. Retrospectively, tumor cells were found in the ...

We report two boys with juvenile dermatomyositis (JDM) complicated by pancreatitis. One also had hepatitis and probably mild bowel vasculitis, while the other had catastrophic bowel vasculitis with multiple perforations. Both were on corticosteroids, but had features of active vasculitis. The former improved with high-dose i.v. pulsed methylprednisolone, while the latter improved only after imm...

RATIONALE Vemurafenib, an inhibitor of mutated B-rapidly accelerated fibrosarcoma, is frequently used in the treatment of melanoma and Erdheim-Chester disease (ECD) patients. Inflammatory adverse effects have been increasingly reported after vemurafenib treatment. PATIENT CONCERNS AND DIAGNOSE We report 6 cases of vemurafenib-associated vasculitis, of whom a personal case of a 75-year-old man...

Both the innate and the acquired immune system are involved in the pathophysiology of renal vasculitis. However, anti-neutrophil cytoplasmic antibody (ANCA)-associated renal vasculitis is characterized by a 'pauci-immune' pattern of immunofluorescence during kidney biopsy, indicating the relative lack of immunoglobulin and complement deposition within the kidney. On the other hand, evidence is ...

Vasculitis is a common and treatable cause of neuropathy. In most cases, necrotizing arteritis of the type observed in polyarteritis nodosa is responsible for the lesions, but classification of vasculitis is still uncertain. The neuropathy often occurs in the context of a multisystem disorder, but in a substantial proportion of patients, especially among patients seen by neurologists, the neuro...

Importance. Infections can cause leukocytoclastic vasculitis. Observations. We report the case of a patient with a left ventricular assist device who presented with acute kidney injury and biopsy proven leukocytoclastic vasculitis. Blood cultures grew Listeria monocytogenes. The patient's rash improved with treatment of the underlying Listeria infection. Conclusion. Clinicians should be aware t...