The syndromes within<unk>  overlap clinically, and patients may have features of more than one disorder. In a retrospective cohort of 136<unk>  patients with systemic involvement from 16 countries, the most prevalent clinical features were fever (84% of cases, often with concurrent constitutional symptoms such as fatigue, malaise, mood disorders or failure to thrive), skin rash (either urticarial or maculopapular rash; 97% of cases) especially after cold exposure, and musculoskeletal involvement (myalgia, arthralgia, and/or arthritis, or less commonly joint contracture, patellar overgrowth, bone deformity, bone erosion and/or osteolytic lesion; 86% of cases). Less common features included ophthalmological involvement (conjunctivitis and/or uveitis, or less commonly optic nerve atrophy, cataract, glaucoma or impaired vision; 71% of cases), neurosensory hearing loss (42% of cases), neurological involvement (morning headache, papilloedema, and/or meningitis, or less commonly seizure, hydrocephalus or mental retardation; 40% of cases), and AA amyloidosis (4% of cases). In keratoendotheliitis fugax hereditaria, systemic symptoms are not reported whereas the patients suffer from<unk> al transient inflammation of the corneal endothelium and stroma, leading to short term blurring of vision and, after repeated attacks, to central corneal stromal opacities in some patients. Age of onset is typically in infancy or early childhood. In 57% of cases,<unk>  had a chronic phenotype with symptoms present almost daily, whereas the remaining 43% of patients experienced only acute episodes. Up to 56% of patients reported a family history of<unk> . Previous studies confirm these symptoms, although the exact reported rates vary.
Cryopyrin-associated periodic syndrome
Hereditary angioedema
Antisynthetase syndrome
Mixed connective tissue disease
IgA vasculitis
TNF receptor associated periodic syndrome
Auto-brewery syndrome
Familial Mediterranean fever