Male with diagnosis of Wegener's granulomatosis associated to anti-proteinase 3 antibodies that improved initially to the treatment with high dose glucocorticoids and ciclophosphamide, but in a relapse he did not have good response to glucocorticoid treatment, ciclophosphamide, methotrexate nor azathioprine. The patient received treatment with rituximab in 4 doses with clinical and radiographic...

BACKGROUND Although gastrointestinal involvement in patients with granulomatosis with polyangiitis is uncommon, it is associated with mild to severe life-threatening complications. We present a case of pneumatosis cystoides intestinalis in a patient with granulomatosis with polyangiitis that was treated successfully with hyperbaric oxygen. CASE PRESENTATION A 70-year-old Japanese man with a 3...

Wegener's disease is one of the lethal forms of necrotizing arteritis. A case involving a young man is presented. The purpose of this report is to inform the Clinicians that a diagnosis of Wegener's granulomatosis or any other arteritis should always be considered in a vague presentation which fails to respond to adequate treatment. The patient's life can be improved and the survival rate in pr...

We report a case of Wegener's Granulomatosis (WG) associated with blastomycosis. This appears to be the first case report of WG co-existing with a tissue proven blastomycosis infection. The temporal correlation of the two conditions suggests that blastomycosis infection (and therefore possibly other fungal infections), may trigger the systemic granulomatous vasculitis in a predisposed individua...

Autoantibodies against components of neutrophil cytoplasm develop during adult vasculitic diseases such as Wegener's granulomatosis and microscopic polyarteritis, and they are predominantly of the IgG class. Similar but distinct antibodies have been described in children with Kawasaki disease and both IgM and IgG class antibodies are represented. This adds another clinically distinct childhood ...

The recent case of necrotising sarcoid granulomatosis described by ALIBERTI et al. [1] provides clinicians with an interesting insight into this rare condition. We would like to supplement the paucity of literature surrounding necrotising sarcoid granulomatosis and illustrate a case of our own. This was also in a young patient but did require treatment with oral corticosteroids at initial prese...

We describe the case of a young patient with a history of non-resolving pneumonia. She was diagnosed with a limited form of Granulomatosis with Polyangiitis (GPA), by percutaneous core needle lung biopsy. In this report, we discuss the definition and clinical implications of limited GPA, treatment options, and highlight the importance of considering vasculitis in the differential diagnosis of n...

Five patients with Wegner's granulomatosis were found to have significantly raised serum immunoglobulin E (IgE) levels. The rise in IgE was not related to the extent of clinical involvement, was not part of a generalized serum immunoglobulin rise, and was not associated with eosinophilia. Raised serum IgE may be a clue to the pathogenesis of this disease.

INTRODUCTION Granulomatosis with polyangiitis poses a significant diagnostic dilemma due its diverse presentations. Seemly isolated sites of disease, such as oral ulcers, may present to physicians working in primary care settings, the emergency room, and subspecialty fields as well as to dentists. Oral presentations are particularly challenging to identify and require a high index of suspicion ...