Adult Kawasaki Disease in a European Patient
Our patient developed the disease in late May. Thorough investigation for many infectious agents did not prove an infectious source of the disease. Nevertheless, he was administered broad-spectrum antibiotics for 7 days. This treatment was not successful and revealed once more that an infection was absent, at least during the patient's hospitalisation. Our differential diagnosis covered a wide range of diseases, mainly infective and autoimmune; none of them could be proved. Given that his condition deteriorated and having no obvious diagnosis available, we performed an extensive review of the current literature, focusing on his symptoms. KD, although rare in adults, could explain the entirety of symptoms and represent a diagnosis of exclusion. Treatment began immediately on the 10th day of the illness, due to suspicion of KD. This was fortunately in contrast to the vast majority of reported adult cases of KD who received treatment long after the 10-day period. In any case, the treatment that was administered would have caused no harm to the patient, even if KD was not present. It is worth noting that even with adequate and timely administration – within ten days – of IVIG and acetylsalicylic acid (ASA), 5% of patients develop coronary abnormalities. The therapeutic result of IVIG supported the diagnosis of KD, since our patient's condition improved considerably within hours.
Our patient did not have some signs and symptoms that are more usual in adult KD, compared to that in children, such as lymphadenopathy and articular involvement. Lymphadenopathy is present in 93% of adult cases of KD, compared to 75% of child cases. Joint problems are observed in 63% of adults with KD, versus 24 to 38% of children with the disease according to a review in 2011.
In contrast, our adult patient had persistent headache that subsided only after the administration of IVIG and ASA. Involvement of the central nervous system takes place more often in children with KD (34%) than in adults (10%).
Our patient's response to the treatment was astonishing and extremely quick: fever resolved and his general condition ameliorated within hours. Such a response is not usual in the KD literature. Most of the patients become afebrile and feel better after 2 days; some others after 1 or 3 days.
Laboratory findings consistent with KD were leucocytosis with neutrophilia, anaemia, late thrombocytosis, elevated ESR and CRP, hypoalbuminemia, hyponatremia, elevated serum transaminases and gamma-glutamyl transpeptidase.
Other than a single IVIG administration and high doses of ASA during the acute phase, no supplementary IVIG or even corticosteroids or immunosuppressive agents were given, since no symptoms persisted or relapsed. ASA was continued for 3 months after the acute phase as an antiplatelet agent. However, some studies suggest immediate discontinuation of all medications after the acute phase, in the absence of CAA. Finally, long-term follow-up was not needed, as no CAA were developed, classifying the patient as risk level I. He was only advised of counselling every 5 years.
Discussion
Our patient developed the disease in late May. Thorough investigation for many infectious agents did not prove an infectious source of the disease. Nevertheless, he was administered broad-spectrum antibiotics for 7 days. This treatment was not successful and revealed once more that an infection was absent, at least during the patient's hospitalisation. Our differential diagnosis covered a wide range of diseases, mainly infective and autoimmune; none of them could be proved. Given that his condition deteriorated and having no obvious diagnosis available, we performed an extensive review of the current literature, focusing on his symptoms. KD, although rare in adults, could explain the entirety of symptoms and represent a diagnosis of exclusion. Treatment began immediately on the 10th day of the illness, due to suspicion of KD. This was fortunately in contrast to the vast majority of reported adult cases of KD who received treatment long after the 10-day period. In any case, the treatment that was administered would have caused no harm to the patient, even if KD was not present. It is worth noting that even with adequate and timely administration – within ten days – of IVIG and acetylsalicylic acid (ASA), 5% of patients develop coronary abnormalities. The therapeutic result of IVIG supported the diagnosis of KD, since our patient's condition improved considerably within hours.
Our patient did not have some signs and symptoms that are more usual in adult KD, compared to that in children, such as lymphadenopathy and articular involvement. Lymphadenopathy is present in 93% of adult cases of KD, compared to 75% of child cases. Joint problems are observed in 63% of adults with KD, versus 24 to 38% of children with the disease according to a review in 2011.
In contrast, our adult patient had persistent headache that subsided only after the administration of IVIG and ASA. Involvement of the central nervous system takes place more often in children with KD (34%) than in adults (10%).
Our patient's response to the treatment was astonishing and extremely quick: fever resolved and his general condition ameliorated within hours. Such a response is not usual in the KD literature. Most of the patients become afebrile and feel better after 2 days; some others after 1 or 3 days.
Laboratory findings consistent with KD were leucocytosis with neutrophilia, anaemia, late thrombocytosis, elevated ESR and CRP, hypoalbuminemia, hyponatremia, elevated serum transaminases and gamma-glutamyl transpeptidase.
Other than a single IVIG administration and high doses of ASA during the acute phase, no supplementary IVIG or even corticosteroids or immunosuppressive agents were given, since no symptoms persisted or relapsed. ASA was continued for 3 months after the acute phase as an antiplatelet agent. However, some studies suggest immediate discontinuation of all medications after the acute phase, in the absence of CAA. Finally, long-term follow-up was not needed, as no CAA were developed, classifying the patient as risk level I. He was only advised of counselling every 5 years.
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