Systemic autoinflammatory diseases are relatively recent entities caused by dysregulation of the innate immune system. They are mainly caused by monogenic mutations, although there are entities produced by polygenic mutations or of multifactorial origin. Traditionally, they have been classified based on the presence or absence of fever, however, thanks to the advancement of knowledge of their Pathogenic mechanisms and the signaling pathways involved, recently it has been advocated to classify them based on the latter. The three more important groups of monogenic autoinflammatory diseases are type 1 interferonopathies, inflammasomopathies and dysregulation in the nuclear factor kappa light chain enhancer of activated B cells [NF-kB] pathway (relopahies). In this review, the main pathways involved, the main syndromes of each of these groups and the therapeutic approach are addressed.

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Myopathies make up an entity that, although rare, can have great clinical significance. These diseases can present a somewhat complex clinical and therapeutic approach due to the etiological versatility and the concomitant symptoms that can sometimes exist. The role of the clinical interview and physical examination is crucial in guiding our diagnostic suspicion. This article will present, in the first instance, the general approach to any type of myopathy in order to request the complementary tests required; and secondly, the clinical characteristics and entities that make up the main groups of myopathies, as well as their therapeutic approach.

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