General Pathology Of Kawasaki Disease

Abstract

Systemic pathological alterations were studied in thirty‐seven autopsied patients with Kawasaki disease. Systemic vasculitis was the most characteristic pathological finding and was present in all the patients. In addition to the vasculitis, there was a high incidence of inflammatory lesions in various organs and tissues: in the heart, endocarditis, myocarditis, and pericarditis; in the digestive system, stomatitis, sialoduct‐adenitis, catarrhal enteritis, hepatitis, cholangitis, pancreatitis, and pancreas ductitis; in the respiratory system, bronchitis and segmental interstitial pneumonia; in the urinary system, focal interstitial nephritis, cystitis, and prostatitis; in the nervous system, aseptic leptomeningitis, choriomeningitis, ganglionitis, and neuritis; in the hematopoietic system, lymphadenitis, splenitis, and thymitis. Dermatitis, panniculitis or myositis were also observed in some patients. Therefore, Kawasaki disease is a systemic inflammatory disease which mainly affects the cardiovascular system. These systemic inflammatory lesions are considered to correspond to the variegated clinical manifestaitions. The relationship between Kawasaki disease and infantile polyarteritis nodosa (IPN) were discussed, based on the clinicopathological characteristics.

The surgical manifestations of the intestinal tract in Kawasaki disease

“…Vasculitis and thrombosis of the small submucosal arteries or submucosa hemorrhage are frequently seen [23,25]. Similar inflammatory changes involving the liver, pancreas, and the lymphoid tissue have also been described [24].…”

“…Surgical manifestations are much less frequent and include small bowel obstruction and strictures [13], intussusception [19], ischemic colitis [12], perforation of duodenum [17], hemorrhagic enteritis [23], appendicitis [18], pancreatitis [9], and hydrops of gallbladder [6]. The typical pathology of the small intestine shows degeneration, necrosis, and desquamation of the epithelial cells, as well as inflammatory exudates in the lamina propria and in the muscular layer [24]. Vasculitis and thrombosis of the small submucosal arteries or submucosa hemorrhage are frequently seen [23,25].…”

Advances in Kawasaki disease

“…In the 1st month after onset of illness, the inflammatory lesions are quite severe. They begin to subside in the 2nd month after onset, and then subside more rapidly in the 3rd month [4]. The pathologic findings in children who had incomplete presentations of KD (''atypical KD''), in whom full diagnostic criteria of KD were not present, are indistinguishable from those seen in classic cases [17].…”

“…Although the likely infectious trigger of the marked inflammatory immune response remains unknown, the pathologic findings have been well-described and are virtually identical in all patients with the illness, varying only in the severity of the lesions. In addition to arteries and veins [3], the following organ systems typically demonstrate inflammatory lesions: cardiovascular system (myocarditis, pericarditis, and less commonly endocarditis); respiratory system (bronchitis, interstitial pneumonia, and pulmonary nodules); digestive system (stomatitis, sialoduct-adenitis, enteritis, hepatitis, cholangitis, pancreatitis, and pancreatic ductitis); urinary system (focal interstitial nephritis, cystitis, and prostatitis); nervous system (aseptic meningitis and neuritis); and hematopoetic system (lymphadenitis and splenitis) [4,16]. In the 1st month after onset of illness, the inflammatory lesions are quite severe.…”

“…Careful study indicates that infantile periarteritis nodosa and fatal KD are pathologically indistinguishable [4,27] and represent the same clinical entity.…”