Discussion:
The extranodal symptoms of KD are uncommon and diverse including skin rash, night sweats, weight loss, headache, cough, and abdominal pain [1]. The disease resolves spontaneously in weeks to months, but in some cases, it may have systemic involvement or evolve to SLE [2-4]. A definite diagnosis of KD is based on characteristic pathologic findings on biopsy that differentiate this disease from others such as lymphoma, SLE, and infectious lymphadenopathy. Characteristic histopathologic findings include necrotic and thrombotic blood vessels. The karyorrhectic foci are formed by different cellular types, predominantly histiocytes and plasmacytoid monocytes, but also immunoblasts and small and large lymphocytes [5].
Recurrence of KD is rare. There have been more recent reports of recurrence rates as high as 13% in an Asian population in Korea. [6] In a study by Jung et al, 54 patients (11.3%) experienced 1–4 recurrent episodes of KD each. The initial recurrence occurred within a mean duration of 6 months (range: 1 month to 6 years). Patients with recurrent KD were more likely to have extranodal symptoms and lymphopenia. Although the etiology of KD recurrence is unknown, certain viral infections have been hypothesized to be among the triggers for KD relapse [8].
Patients with KD showing progression to autoimmune diseases were more likely to have fever, common extranodal symptoms, a higher recurrence rate, and a higher ANA positivity rate at KD diagnosis [1]. Hepatosplenomegaly was present in 18% of patients of KD [7] with few cases having clinically diagnosed AIH. Our case had biopsy proven AIH without clinical and laboratory findings of SLE. In addition, our case had recurrence of symptoms of KD after 9 years which improved after starting corticosteroids.