Adult Kawasakiʹs disease with myocarditis, splenomegaly, and highly elevated serum ferritin levels

Kawasakiʹs disease is a disease of unknown cause. The characteristic clinical features of Kawasakiʹs disease are fever ≥ 102°F for ≥ 5 days accompanied by a bilateral bulbar conjunctivitis/conjunctival suffusion, erythematous rash, cervical adenopathy, pharyngeal erythema, and swelling of the dorsum of the hands/feet. Kawasakiʹs disease primarily affects children and is rare in adults. In children, Kawasakiʹs disease is more likely to be associated with aseptic meningitis, coronary artery aneurysms, and thrombocytosis. In adult Kawasakiʹs disease, unilateral cervical adenopathy, arthritis, conjunctival suffusion/conjunctivitis, and elevated serum transaminases (serum glutamic oxaloacetic transaminase [SGOT]/serum glutamate pyruvate transaminase [SGPT]) are more likely. Kawasakiʹs disease in adults may be mimicked by other acute infections with fever and rash, that is, group A streptococcal scarlet fever, toxic shock syndrome (TSS), and Rocky Mountain Spotted Fever (RMSF). Because there are no specific tests for Kawasakiʹs disease, diagnosis is based on clinical criteria and the syndromic approach. In addition to rash and fever, scarlet fever is characterized by circumoral pallor, oropharyngeal edema, Pastiaʹs lines, and peripheral eosinophilia, but not conjunctival suffusion, splenomegaly, swelling of the dorsum of the hands/feet, thrombocytosis, or an elevated SGOT/SGPT. In TSS, in addition to rash and fever, there is conjunctival suffusion, oropharyngeal erythema, and edema of the dorsum of the hands/feet, an elevated SGOT/SGPT, and thrombocytopenia. Patients with TSS do not have cervical adenopathy or splenomegaly. RMSF presents with fever and a maculopapular rash that becomes petechial, first appearing on the wrists/ankles after 3 to 5 days. RMSF is accompanied by a prominent headache, periorbital edema, conjunctival suffusion, splenomegaly, thrombocytopenia, an elevated SGOT/SGPT, swelling of the dorsum of the hands/feet, but not oropharyngeal erythema. sent a case of adult Kawasakiʹs disease with myocarditis and splenomegaly. The patientʹs myocarditis rapidly resolved, and he did not develop coronary artery aneurysms. In addition to splenomegaly, this case of adult Kawasakiʹs disease is remarkable because the patient had highly elevated serum ferritin levels of 944-1303 ng/mL; (normal < 189 ng/mL). To the best of our knowledge, this is the first report of adult Kawasakiʹs disease with highly elevated serum ferritin levels. This is also the first report of splenomegaly in adult Kawasakiʹs disease. We conclude that Kawasakiʹs disease should be considered in the differential diagnosis in adult patients with rash/fever for ≥ 5 days with conjunctival suffusion, cervical adenopathy, swelling of the dorsum of the hands/feet, thrombocytosis and otherwise unexplained highly elevated ferritin levels.