Kawasaki disease in adults: Observations in France and literature review.

OBJECTIVE: Kawasaki disease (KD) is a vasculitis that mostly occurs in young children and rarely in adults. We analyzed the characteristics of adult-onset KD (AKD) in France. METHODS: We collected retrospective and prospective data for patients with a diagnosis of KD occurring after the age of 18 years. Cases were obtained via various French medical networks and identified from the international literature. RESULTS: We included 43 patients of AKD at 26 institution from 1992 to 2015, with mean (SD) age 30 (11) years (range 18-68) and sex ratio (M/F) 1.2; 34 patients met the American Heart Association criteria and 9 were incomplete AKD. The median time to diagnosis was 13 days (interquartile range 8-21). The main symptoms were fever (100%), exanthema (98%), changes in the extremities (91%), conjunctivitis (77%), oral cavity changes (89%), cervical adenitis (55%) and cardiac abnormalities (45%). Overall, 35% of patients showed large-vessel vasculitis: coronary vasculitis (26%) and coronary aneurysm (19%). Treatment was mostly intravenous immunoglobulins (79%) and aspirin (81%). Four patients showed myocardial infarction due to coronary vasculitis, but none were treated with IVIg because of late diagnosis. After a median follow-up of 5 months (range 1-117), persistent aneurysm was noted in 9% of cases. Damage was significantly lower with early treatment than late or no treatment (p=0.01). CONCLUSION: Given the high frequency of cardiac involvement and complications in this series of AKD, diagnosis and treatment should not be delayed, and early IVIg treatment seems to improve the outcome.

Kawasaki disease in adults: report of 10 cases.

Kawasaki disease (KD) is an acute multisystemic vasculitis occurring predominantly in children and rarely in adults. Diagnosis is made clinically using diagnostic guidelines; no specific test is available. "Incomplete" KD is a more recent concept, which refers to patients with fever lasting > or =5 days and 2 or 3 clinical criteria (rash, conjunctivitis, oral mucosal changes, changes of extremities, adenopathy), without reasonable explanation for the illness. To describe the clinical and laboratory features of classical (or "complete") KD, and incomplete KD in adults, we report 10 cases of adult KD, including 6 patients who fulfilled the criteria for incomplete KD, diagnosed either at presentation (n = 4) or retrospectively (n = 2). At the time of clinical presentation, complete KD was diagnosed in 4 patients, while 4 patients fulfilled the criteria for incomplete KD. For 3 of the 4 patients with incomplete KD, presence of severe inflammation, laboratory findings (hypoalbuminemia, anemia, elevation of alanine aminotransferase, thrombocytosis after 7 days, white blood cell count > or =15,000/mm, and urine > or =10 white blood cell/high power field), or echocardiogram findings were consistent with the diagnosis. In 2 patients, the diagnosis of KD was made retrospectively in the presence of myocardial infarction due to coronary aneurysms, after an undiagnosed medical history evocative of incomplete KD. Seven patients received intravenous immunoglobulins (IVIG), after a mean delay of 12.5 days, which appeared to shorten the course of the disease. This relatively large series of adult KD highlights the existence of incomplete KD in adults and suggests that the algorithm proposed by a multidisciplinary committee of experts to diagnose incomplete KD in children could be useful in adults. Further studies are needed to determinate whether prompt IVIG may avoid artery sequelae in adult patients with complete or incomplete KD.

Clinical significance of anti-histidyl-tRNA synthetase (Jo1) autoantibodies.

The clinical significance of a discovery of anti-histidyl-tRNA synthetase (Jo1) autoantibodies patients was established in the early diagnosis of antisynthetase syndrome (ASS) as the common form of this pathology is characterized by interstitial lung disease (ILD), inflammatory muscle disease, and production of anti-Jo1 autoantibodies. However, the specificity of such autoantibodies has to be evaluated in daily clinical practice. In this study, the clinical and prognostic profiles of 45 patients displaying anti-Jo1 autoantibodies were determined. Among 36 patients with a titer of anti-Jo1 autoantibodies above the cutoff value suggested by the manufacturer (40 AU/mL), three different groups were identified. The first group (n = 26) suffered from a complete or incomplete ASS and showed anti-Jo1 autoantibodies mostly above 60 AU/mL. A second group (n = 7) suffered from another autoimmune disease, that is, a systemic lupus erythematosus, cutaneous lupus and rheumatoid arthritis, and Crohn's disease with anti-Jo1 autoantibodies mostly below 60 AU/mL. The third group (n = 3) did not suffer from any autoimmune disease and presented anti-Jo1 autoantibodies below 60 AU/mL. The nine doubtful cases (titer of anti-Jo1 autoantibodies of 30-39 AU/mL) were from patients with no ASS nor myositis. Only 27 out of 45 patients showed antinuclear antibodies with 15 sera showing a pattern characteristic of anti-Jo1 autoantibodies by indirect immunofluorescence on HEp2 cells. In conclusion, this study underlines the need to search for anti-Jo1 autoantibodies even if antinuclear antibodies are negative by indirect immunofluorescence and underlines the usefulness of anti-Jo1 antibodies of titer above 60 AU/mL in the diagnosis of complete or incomplete ASS.