Kimuras disease is a benign rare chronic inflammatory disorder. Its primary symptoms are subdermal lesions in the head or neck or painless unilateral inflammation of cervical lymph nodes. Cause
Its cause remains unknown. Reasons such as an allergic reaction, tetanus toxoid vaccination, or an alteration of immune regulation are suspected. Other theories like persistent antigenic stimulation following arthropod bites and parasitic or candidal infection have also been proposed. To date, none of these theories has been substantiated. Pathophysiology
The pathophysiology of Kimuras disease remains unknown, although an allergic reaction, trauma, and an autoimmune process have all been implicated as possible causes. The disease is manifested by an abnormal proliferation of lymphoid follicles and vascular endothelium. Peripheral eosinophilia and the presence of eosinophils in the inflammatory infiltrate suggest it may be a hypersensitivity reaction. Some evidence has indicated TH2 lymphocytes may also play a role, but further investigation is needed.Kimuras disease is generally limited to the skin, lymph nodes, and salivary glands, but patients with Kimuras disease and nephrotic syndrome have been reported. The basis of this possible association is unclear. Diagnosis
An open biopsy is the chief means by which this disease is diagnosed. "Lymphoid nodules with discrete germinal centers can occupy an area extending from the reticular dermis to the fascia and muscle. Follicular hyperplasia, marked eosinophilic infiltrate and eosinophilic abscesses, and the proliferation of postcapillary venules are characteristic histological findings. [18] Centrally, thick-walled vessels are present with hobnail endothelial cells. Immunohistochemical evaluation of the lymphoid nodules demonstrates a polymorphous infiltrate without clonality.
Thus VUR not only increases the frequency of UTIs, but also the risk of damage to upper urinary structures and end-stage renal disease. Severity
Vesicoureteral reflux (VUR) is graded according to severity. Grade I – reflux into non-dilated ureter
Grade II – reflux into the renal pelvis and calyces without dilatation
Grade III – mild/moderate dilatation of the ureter, renal pelvis and calyces with minimal blunting of the fornices
Grade IV – dilation of the renal pelvis and calyces with moderate ureteral tortuosity
Grade V – gross dilatation of the ureter, pelvis and calyces; ureteral tortuosity; loss of papillary impressionsThe younger the patient and the lower the grade at presentation the higher the chance of spontaneous resolution. Approximately 85% of grade I & II VUR cases will resolve spontaneously. Approximately 50% of grade III cases and a lower percentage of higher grades will also resolve spontaneously. Treatment
The goal of treatment is to minimize infections, as it is infections that cause renal scarring and not the vesicoureteral reflux. Minimizing infections is primarily done by prophylactic antibiotics in newborns and infants who are not potty trained. However, in children who are older, physicians and parents should focus on bowel and bladder management. Children who hold their bladder or who are constipated have a greater number of infections than children who void on a regular schedule. When medical management fails to prevent recurrent urinary tract infections, or if the kidneys show progressive renal scarring then surgical interventions may be necessary.