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Kawasaki disease (KD) is an immune mediating vasculitis affecting many systems especially the cardiovascular system. Pirfenidone is known for its anti-fibrotic and anti-inflammatory effects.Aim of work: to study the possible protective effect of pirfenidone on cardiac complications in a model of KD induced by Bacillus Calmette-Gue´rin (BCG) injection.Materials and Methods: This study included 18 female Balb/C mice that were divided into three groups. Group I (control group), group II (KD group) that received single injection of BCG in tail vein, and group III (pirfenidone group) that received BCG as group II and daily oral pirfenidone till end of experiment. All mice were sacrificed 21 days after the injection, hearts were collected and examined.Results: Group II revealed irregular separated cardiac myocytes and interstitial inflammation. Coronaries were seen with thin wall and irregular lumen. Localized intimal thickening and irregularly arranged smooth muscles were noticed in the media. Perivasular inflammation was also noticed. Mallory stained sections revealed interstitial, vascular and perivascular fibrosis. Orcein stained sections revealed disruption of internal and external elastic laminae. Positive immune reaction for TNF-α was also noticed in group of KD. Pirfenidone treatment minimized the histological changes induced in KD. Little mononuclear cellular infiltration was still noticed in the myocardium. Moderate collagen fibers were seen in the adventitia and intact elastic laminae.Conclusions: Pirfenidone aborted the cardiovascular complications associated with KD.

Effect of pirfenidone on cardiac complications in a model of Kawasaki disease in female Balb/C Mice: Histological and Immunohistochemical study