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Background: Microscopic polyangiitis (mPA) is one of the ANCA-associated prim ary systemic vasculitides. It is defined as necrotic vasculitis without granulom a development and with-out/minimal immunodepots in situ. The clinical picture i s characterised by destruction of the small vessels with the main manifestations in the upper and lower respiratory tract, in the lungs and kidneys. The eye man ifestations were noted yet not so often. Patient: There were 2 clinical cases, a ged 5-12 years, with primary eye manifestations of the mPA. The initial oedema and the reddening of the lid area were common to all the patients. The diagnosti c excisions of the inflammatory tumour of the anterior orbital area have shown h istologically vasculitis of the small vessels. mPA was diagnosed by clinical and immunological parameters, including the examination of the respiratory tract an d kidneys. Conclusions: In inflammatory orbital pseudotumour, diagnostic excisio n and evaluation of the immunological parameters are useful for the exclusion of a systemic vasculitis. Proper examinations must be performed to determine other possible organ manifestations. Prognosis is characterised by the severity of ki dney involvement. Early immunosuppressive therapy is crucially important for sur vival prognosis.
Background: Microscopic polyangiitis (mPA) is one of the ANCA-associated priminy systemic vasculitides. It is defined as necrotic vasculitis without granulo a development and with-out / minimal immunodepots in situ. The clinical picture is characterised by destruction of the small vessels with the main manifestations in the upper and lower respiratory tract, in the lungs and kidneys. The eye man ifestations were noted yet not so often. Patient: There were 2 clinical cases, a ged 5-12 years, with primary eye manifestations of the mPA. The initial oedema and the reddening of the lid area were common to all the patients. The diagnosti c excisions of the inflammatory tumor of the anterior orbital area have shown h isologicallyologically vasculitis of the small vessels. mPA was diagnosed by clinical and immunological parameters , including the examination of the respiratory tract an d kidneys. Conclusions: In inflammatory orbital pseudotumor, diagnostic excisio n and evaluation of the immunologica l parameters are useful for the exclusion of a systemic vasculitis. Proper examinations must be performed to determine other possible organ manifestations. Prognosis is characterised by the severity of ki dney involvement. Early immunosuppressive therapy is crucially important for sur vival prognosis.