Life-threatening complications of adult-onset Still’s disease

Abstract

Adult-onset Still's Disease (AOSD) since its description in 1971 has proven to be a very complex and challenging disease entity. This rare auto-inflammatory disease is classically described by the "Still's triad" of fever, rash, and arthritis, although the atypical cases frequently outnumber the typical ones. The exact pathogenesis and etiologic factors responsible for the clinical features remain largely obscure, despite recent suggestive cytokine biology findings. Diagnosis is made on clinical grounds, following the exclusion of mimickers of infectious, autoimmune or neoplastic etiology, with the additional consideration of non-specific laboratory abnormalities such as peripheral leukocytosis and elevation of serum ferritin and other acute phase reactants. The disease manifestations are protean and can include diverse complications, affecting multiple organ systems. Moreover, the severity of the organ involvement can vary considerably, representing a wide spectrum from the self-limited to severe. The mainstay of therapy has evolved from the traditional use of corticosteroids and oral immunosupressants to the newer targeted treatments with biologic agents. The scope of this review is to alert the clinician to the existence of life-threatening AOSD complications, namely the macrophage activation syndrome, disseminated intravascular coagulopathy, thrombotic thrombocytopenic purpura, diffuse alveolar hemorrhage, and pulmonary arterial hypertension. Such knowledge may lead in earlier recognition, prompt treatment, and, ideally, improved patient outcomes.

Pathogenesis of adult-onset Still’s disease: new insights from the juvenile counterpart

“…Furthermore, overt reHLH has been shown to complicate 7-10 and 12-17 % of sJIA and AOSD, respectively [15,24,71]. Evidence for occult reHLH in a substantial proportion of patients with sJIA or AOSD (up to 50 %) supports the possibility that HLH and sJIA/AOSD could represent a continuum of severity [72,73].…”

Presentation and diagnosis of adult-onset Still’s disease: the implications of current and emerging markers in overcoming the diagnostic challenge

“…Recently, Efthimiou et al [33] identified some unfavorable prognostic markers of disease, which should make the clinicians aware of a possible negative evolution. Such cases are more prone to become refractory to treatment over the course of disease.…”

“…The most frequent complications of the disease were disseminated intravascular coagulation, macrophage activation syndrome (MAS), acute respiratory distress syndrome, myocarditis (sporadically complicated by cardiac tamponade) and liver failure. In general, MAS is one of the most feared life-threatening complications with a reported mortality rate ranging between 10 and 22% [33]. MAS should be increasingly suspected in light of the presence of high fever, lymphadenopathy, hepatosplenomegaly, pancytopenia, high serum levels of ferritin, triglycerides and liver enzymes but with normal erythrocyte sedimentation rate (ESR).…”

A Case of Atypical Urticaria

“…A few cases have been reported in the literature in which AOSD manifested with atypical cutaneous features, causing a delay in diagnosis (6, 7 ). In some cases, this led to serious complications such as HLH, disseminated intravascular coagulopathy, thrombotic thrombocytopenic purpura, diffuse alveolar hemorrhage, and pulmonary arterial hypertension (8 ).…”