Search results
Results from the WOW.Com Content Network
The full clinical picture was first presented by Friedrich Wegener (1907–1990), a German pathologist, in two reports in 1936 and 1939, leading to the eponymous name Wegener's granulomatosis or Wegener granulomatosis (English: / ˈ v ɛ ɡ ə n ər /). [10]
Subsequent studies found that the cells infiltrating the midline tissues in cases of lethal midline granuloma that were not clearly diagnosed as granulomatosis with polyangiitis were: a) infected by the Epstein–Barr virus [2] and b) consisted of malignant lymphocytes, usually NK cells or, rarely, cytotoxic T cells. [3]
Eosinophilic granulomatosis with polyangiitis consists of three stages, but not all patients develop all three stages or progress from one stage to the next in the same order; [7] whereas some patients may develop severe or life-threatening complications such as gastrointestinal involvement and heart disease, some patients are only mildly affected, e.g. with skin lesions and nasal polyps. [8]
The negative immunofluorescence pattern, however, is called "pauci-immune" and is often associated with systemic vasculitides (plural of vasculitis) including: microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis (EGPA), and granulomatosis with polyangiitis (GPA). [1] [2]
Granulomatosis with polyangiitis, formerly known as Wegener's granulomatosis, is a rare immune-mediated systemic disease with an unclear etiology. It manifests pathologically as an inflammatory response pattern in the kidneys, upper and lower respiratory tracts , and granulomatous inflammation, which includes necrosis .
The additional cells are sometimes a clue to the cause of the granuloma. For example, granulomas with numerous eosinophils may be a clue to coccidioidomycosis or allergic bronchopulmonary fungal disease, and granulomas with numerous neutrophils suggest blastomycosis, granulomatosis with polyangiitis, aspiration pneumonia, or cat-scratch disease.
p-ANCA is associated with several medical conditions: [3] It is fairly specific, but not sensitive for ulcerative colitis, so is not useful as a sole diagnostic test. [4] When measured together with anti-saccharomyces cerevisiae antibodies (ASCA), p-ANCA has been estimated to have a specificity of 97% and a sensitivity of 48% in differentiating patients with ulcerative colitis from normal ...
Granulomatosis with polyangiitis: Crusting rhinorrhea, sinusitis, chronic otitis media, nasal obstruction, shortness of breath, and chronic cough. [15] [16] Eosinophilic granulomatosis with polyangiitis: Asthma, allergic rhinitis, sinusitis, nasal polyps, peripheral neuropathy, pulmonary infiltrates, and abdominal pain. [17] [18]