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The anti–interleukin-1 antibody canakinumab was effective at controlling and preventing recurrence of flares in autoimmune inflammatory diseases: familial Mediterranean fever, mevalonate kinase deficiency, and the TNF receptor–associated periodic syndrome.

Autoinflammatory diseases are characterized by fever attacks of varying durations, associated with variety of symptoms including abdominal pain, lymphadenopathy, polyserositis, arthritis, etc. Despite the diversity of the clinical presentation, there are some common features that make the differential diagnosis of the autoinflammatory diseases challenging. Familial Mediterranean fever (FMF) is the most commonly seen autoinflammatory conditions, followed by syndrome associated with periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA). In this review, we aim to evaluate disease characteristics that make a diagnosis of FMF and PFAPA challenging, especially in a regions endemic for FMF. The ethnicity of patient, the regularity of the disease attacks, and the involvement of the upper respiratory systems and symphonies could be helpful in differential diagnosis. Current data from the literature suggest the use of biological agents as an alternative for patients with FMF and PFAPA who are non-responder classic treatment options. More controlled studies are needed to evaluate the efficacy and safety of this strategy.

Adenosine deaminase 2 (ADA2) is a protein with at least two functions. It is a growth factor affecting leukocytes and endothelial cells and an enzyme that influences purine metabolism. This study shows that mutant ADA2 causes polyarteritis nodosa. Polyarteritis nodosa, first described in 1866, 1 is a systemic necrotizing vasculitis that affects medium and small muscular arteries. 2 , 3 The ensuing tissue ischemia can affect any organ, including the skin, musculoskeletal system, kidneys, gastrointestinal tract, and the cardiovascular and nervous systems. Polyarteritis nodosa is usually diagnosed in middle age or later but can appear in childhood. 2 , 4 , 5 The diagnosis remains challenging despite classification criteria for adults 6 and children, 7 because polyarteritis nodosa frequently presents with nonspecific constitutional symptoms, and organ involvement and disease severity are highly varied. Polyarteritis nodosa is most often primary, but in adults it may be associated . . .

In two placebo-controlled trials, canakinumab, an anti-interleukin-1β monoclonal antibody, achieved a response, prevented flares, and allowed glucocorticoid tapering in patients with systemic juvenile idiopathic arthritis. Infection was more common with canakinumab than with placebo. Systemic juvenile idiopathic arthritis (JIA), the most severe JIA subtype, is characterized by chronic arthritis; intermittently high, spiking temperatures; maculopapular rash; hepatosplenomegaly; lymphadenopathy; serositis; and a marked increase in the level of acute-phase reactants. 1 – 3 Complications of systemic JIA include growth impairment, osteoporosis, and the potentially lethal macrophage activation syndrome. 4 – 6 Until recently, systemic JIA was considered a therapeutic orphan, since the principal effective treatment was glucocorticoids, with their known toxicity and long-term growth and bone sequelae. 7 Other therapeutic options include nonsteroidal antiinflammatory drugs (NSAIDs), methotrexate, and biologic agents. Both interleukin-6 8 – 10 and, more recently, interleukin-1 11 – 14 have been found . . .

Concerns regarding the comorbidity as a significant risk factor for Coronavirus Disease-2019 (COVID-19), gave rise to an urgent need for studies evaluating patients with chronic conditions such as autoinflammatory diseases (AIDs). We prepared a web-based survey investigating the clinical findings and contact histories among pediatric patients with AIDs. Confirmed COVID-19 cases, patients with contact history and those with symptoms which were highly suggestive of COVID-19 were called via phone or recruited to a video or face to face appointment. Data of AIDs were obtained from their medical records, retrospectively. Laboratory and screening findings were confirmed by our national health registry website. There were 404 patients (217 female) eligible for the enrollment. During pandemic, 375 (93%) were on colchicine treatment and 48 (11.8%) were receiving biologic treatment. Twenty-four out of 404 patients were admitted to hospital due to COVID-19 suspicion. Severe acute respiratory syndrome coronavirus-2 (SARS CoV-2) was identified through rhinopharyngeal swabs in seven patients, six of whom were only on colchicine treatment. Only one patient with no finding of any severe respiratory complications was hospitalized. All of seven patients recovered completely. Among patients on biologic drugs, neither a symptom nor a positive polymerase chain reaction test for COVID 19 was detected. In conclusion, pediatric patients with AIDs, those receiving biologic treatment and/or colchicine, may not be at increased risk for neither being infected nor the severe disease course.