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Idiopathic inflammatory myopathies (IIMs) are a diverse group of diseases characterized by proximal muscle weakness and inflammation in skeletal muscle. Phenotypically, the subtypes include dermatomyositis, polymyositis, inclusion body myositis, and amyopathic dermatomyositis. The most common IIM in children is juvenile dermatomyositis (JDM). In contrast to adult dermatomyositis (DM), children are likely to have frequent relapses, vasculopathy, and long-term metabolic and other complications like lipodystrophy, insulin resistance, and calcinosis. Significant advances in our understanding of pathogenesis, disease course, and treatment of JDM has changed the therapeutic landscape and improved outcomes in children. Myositis-specific autoantibodies and myositis-associated autoantibodies have unique clinical associations, disease course and help predict response to therapy. A multidisciplinary approach including exercise programs and psychosocial support is essential. The first line of treatment is a combination of corticosteroids and methotrexate (MTX). Other targeted immunosuppressive therapy is used in refractory cases. Early recognition and timely referral to a specialist center remain pivotal to improving the mortality and morbidity associated with this disease.

Autoinflammatory diseases affecting the NLRP3 gene are rare autosomal dominant disorders presenting with episodic organ limited and systemic inflammation. We report three patients with cryopyrinopathies. Our first case is a 4-year-old boy with a history of periodic fever, failure to thrive, and raised intracranial pressure. The second case is a 6-year-old boy with similar complaints, also with bilateral uveitis. The third is a 24-year-old gentleman with periodic fever and early hearing loss, also with a novel presentation of sacroiliitis. Our case series demonstrates that there should be a low clinical threshold indicating genetic testing in any child who displays features of autoinflammation in combination with an urticarial rash, musculoskeletal manifestations, hearing loss, and chronic aseptic meningitis with macrocephaly. Furthermore, despite anakinra being a cornerstone in treating NLRP-3 AID, there is an unmet clinical need to provide access to alternatives such as colchicine and thalidomide in resource-limited settings.