A case report of rapidly progressive juvenile dermatomyositis complicated by sepsis

Oshlyanska O.A.^1,2, Stepaniuk Y.S.³, Rudenko S.M.³, Leibiuk Y.V.⁴, Shatrova К.М.¹, Savostikova N.L.³, Slobodian S.V.³, Synoverska O.B.^4,5

• ¹Shupyk National Healthcare University of Ukraine
• ²State Institution «Ukrainian Center of Maternity and Childhood of the National Academy of Medical Sciences of Ukraine»
• ³National Specialized Children’s Hospital «Okhmatdyt» of the Ministry of the Health of Ukraine
• ⁴Ivano-Frankivsk Regional Children’s Clinical Hospital
• ⁵Ivano-Frankivsk National Medical University

Summary. The article presents a case report of rapidly progressive juvenile dermatomyositis (JDM) in an adolescent complicated by sepsis and duodenal perforation. Despite the useof modern treatment methods, including immunobiological therapy, mortality among these patients remains quite high (about 3%). Diagnostic difficulties, severe myopathic syndrome at the onset of the disease, interruption of treatment and development of secondary immunodeficiency on the background of anti-B lymphocyte therapy have become key factors in the unfavourable prognosis. Typical mistakes and problems in the management of patients with severe JDM are highlighted, the importance of monitoring the immune status and continuous treatment control at all stages of management of such patients is emphasised.