The protracted superficial phenomenon in pathergic (Wegener's) granulomatosis**
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Antineutrophil cytoplasmic antibody-negative granulomatosis with polyangiitis localized to the lungs
2022, Respiratory Medicine Case ReportsCitation Excerpt :The second reason is that localized GPA may simply be the early stage in the clinical course of the disease. Although it is well known that GPA can show initially indolent course [16,17], localized and non-life-threatening GPA can relapse and extend to generalized GPA despite a combination of immunosuppressants. A prospective, long-term, observational study demonstrated that 10% of patients with localized GPA progressed to the systemic form and 46% had relapse even after immunosuppressant therapy [5].
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2010, Rheumatic Disease Clinics of North AmericaCitation Excerpt :In biopsy specimens of such masses, granuloma formation can be detected in addition to vasculitis and geographic necrosis. It has been hypothesized that WG starts with inflammation in the upper and lower respiratory tract characterized by granulomatous lesions and progresses to ANCA-associated small-vessel vasculitis in subsequent stages.47 According to this hypothesis, an aberrant immune response may be triggered by an unknown bacterial antigen (eg, S aureus) leading to formation of granulomatous lesions and a subsequent adaptive immune response against PR3 (PR3-ANCA production).
The vasculopathic reaction pattern
2009, Weedon's Skin Pathology: Third EditionTrastuzumab-induced cytoplasmic anti-neutrophilic cytoplasmic antibody necrotizing granulomatous panniculitis
2006, Journal of the American Academy of DermatologyWegener's granulomatosis presenting as acute systemic vasculitis following 20 years of limited tracheobronchial disease
2009, Journal of Laryngology and Otology
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Presented at the 13th International Congress, International Academy of Pathology, Paris, France, September 16, 1980.
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Consultant in Pathology, Massachusetts General Hospital, Boston, Massachusetts.