Kawasaki disease (KD) is a systemic inflammatory condition of uncertain origin that predominantly affects children under five years of age. The exact cause of Kawasaki disease remains unknown, although seasonal episodes, particularly in summer and winter, suggest the possibility of an infectious trigger. Diagnostic criteria for adult Kawasaki disease have also been proposed [5].
The diagnosis is probable if an individual presents with unexplained persistent fever for more than 5 days with an onset age of first manifestations over 18 years, along with coronary involvement and 3 out of 5 criteria, which are as follows: i) exanthema; ii) oral mucosal changes; iii) extremity changes; iv) conjunctivitis; v) lymphadenopathy. Our patient met four criteria in addition to prolonged fever and coronary involvement, which was sufficient to establish a diagnosis of Kawasaki disease.
Pericarditis is a common but under-reported manifestation. It is typically mild and asymptomatic [6]. Digestive involvement was also possible. Common gastrointestinal signs include abdominal pain, diarrhea, hepatitis, vomiting, and gallbladder hydrops; jaundice and pancreatitis are less common [7]. Histological pancreatic involvement has been commonly observed in autopsy reports, but clinical pancreatitis remains rare [8]. Pancreatitis in Kawasaki disease is characterized by vasculitis of the medium-sized arteries and veins. The beneficial effects of IV Ig in Kawasaki disease complicated by pancreatitis have been previously explained by the resolution of vasculitis in pancreatic blood vessels [9]. A literature review highlighted that pancreatitis can precede the onset of Kawasaki disease symptoms, be part of the clinical picture of the disease, or even occur several days after IgIV administration. Abdominal pain is the most common symptom of pancreatic involvement, although some patients remain asymptomatic and only exhibit elevated lipase levels [10]. Only two cases of Kawasaki disease in adults with pancreatitis have been reported in the literature [8, 11], and to date, no cases have been described in adults presenting with both pancreatitis and coronary artery aneurysm.
The recommended treatment regimen for Kawasaki disease is a single infusion of 2 g/kg of IVIG, which may be repeated in cases of resistance defined by persistent or recurrent fever after 36 h [3]. Treatment should be initiated promptly, preferably before the 10th day following the onset of the disease, as long as the inflammation persists. However, diagnosis within the first 10 days of the disease is rare and often results in delayed treatment initiation [12]. Corticosteroids are commonly prescribed for most vasculitides because of their rapid action, potent anti-inflammatory properties, and generally positive outcomes. Their use in Kawasaki disease treatment is more debated, but emerging data suggest that patients at high risk of developing coronary artery aneurysms may benefit from early initiation of corticosteroids in addition to IVIG and aspirin [1]. Although the diagnosis was made 20 days after the onset of the first symptoms, a bolus of methylprednisolone was administered to our patient because of the presence of two severe manifestations, pancreatic and cardiac, in combination with immunoglobulins and aspirin.