M.A. AFANASYEVA¹, E.V. DYAKOVA¹, G.R. GAINULLINA², R.Z. ABDRAKIPOV¹, N.L. NOVICHKOVA², S.A. LAPSHINA², D.I. ABDULGANIYEVA²

¹Republic Clinical Hospital of the MH of RT, 138 Orenburgskiy  Trakt, Kazan, Russian Federation, 420064

²Kazan State Medical University, 49 Butlerov Str., Kazan, Russian Federation, 420012

 Afanasyeva M.A. ― doctor of the Rheumatology Department, tel. (843) 231-21-67, e-mail: afmaria2108@mail.ru

Dyakova E.V. ― doctor of the Pulmonology Department, tel. (843) 231-21-58, e-mail: vrkaty.dyakowa@yandex.ru

Gainullina G.R. ― resident doctor of the Department of Hospital Therapy, tel. +7-937-529-72-38, e-mail: nice.gaynullina@bk.ru

Abdrakhipov R.Z. ― Head of the Rheumatology Department, tel. (843) 231-21-67, e-mail: hosptherapy@mail.ru

Novichkova N.L. ― resident doctor of the Department of Hospital Therapy, tel. (843) 231-21-67, e-mail: novichkovanl@yandex.ru

Lapshina S.A. ― Cand. Med. Sc., Assistant of the Department of Hospital Therapy, tel. (843) 237-34-83, e-mail: svetlanalapshina@mail.ru

Abdulganiyeva D.I. ― D. Med. Sc., Professor, Head of the Department of Hospital Therapy, tel. (843) 237-34-83, e-mail: diana_s@mail.ru

 Systemic vasculites (SVs) are characterized by inflammation of blood vessels wall; the spectrum of their clinical manifestations depends on the type, extent, and location of affected vessels and the activity of systemic inflammation. The subgroup of systemic necrotizing vasculitis associated with the synthesis of ANCA includes microscopic polyangiitis (MPA), mainly affecting small vessels of the lungs and kidneys. A case is presented of difficult diagnostic search in a young patient with a rapidly progressive variant of development of systemic vasculitis under the mask of SLE and a severe complication in the form of hemoptysis in the debut. The disease occurred with damage of joints and lungs (pulmonitis, recurrent hemoptysis, interstitial pulmonary edema). Despite intensive pulse therapy with corticosteroids clinical response was not received. Rituximab (RTM) was prescribed. Its effectiveness is shown with relief of the main symptoms and the achievement of clinical and laboratory remission.

Key words: systemic vasculitides, microscopic polyangiitis, diagnosis, treatment.

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