Kimura's Disease: A Rare Cause of Concurrent Lymphadenopathy and Nephrotic Syndrome

To the Editor

One rare cause of lymphadenopathy and concurrent nephrotic syndrome that is often overlooked is Kimura's disease (KD). KD or “eosinophilic lymphoid granuloma” involves chronic lymphoid inflammatory changes and was first reported by Kimm and Szeto in 1937. It is more common in the Asian subContinent. ¹ Mean age of patients is 22.8 years, ² although it has been reported in patients as young as 9 years of age. A higher preponderance of the disease is seen in males. The exact etiology of KD remains unclear. Interestingly, nearly 60% of patients with KD develop renal pathologies. ³ Rarely, the pulmonary system may be involved. For instance, Koh et al. recently reported the case of a 51-year-old male with KD who developed eosinophilic lung disease. ⁴

Patients present with submandibular subcutaneous swellings in 75% of the cases. ⁵ The swellings are typically painless. The pre-auricular area is also affected often. Edema is the presenting symptom in 25% of patients. Parotid masses may develop in a few patients. Twelve percent of patients with KD exhibit proteinuria. ⁶ Nephrotic syndrome is seen in nearly half of these cases. Nephrotic syndrome tends to be more common in adults with KD. The nephrotic syndrome may precede the appearance of lymphatic changes by as much as 3 years. Interestingly, many rare forms of glomerulonephritis have been reported in KD. For instance, Chauvel et al. recently reported a patient with KD who developed “necrotic extra-capillary glomerulonephritis”. ⁷ Renal failure may develop in a few patients, resulting in the necessity of chronic dialysis.

Peripheral eosinophilia is seen in 88.8% of patients. ⁸ Viswanatha et al. in a recent study also reported accentuated Ig-E levels in 83.3% of patients. ⁹ Lymph node biopsies usually reveal hyperplastic lymphoid follicles with germinal centers. Eosinophilic infiltration of the para-cortical areas is seen in addition. Post-capillary venular proliferation may also be present simultaneously. Renal biopsies usually reveal mesangial proliferation with concurrent fusion of the epithelial foot processes. Eosinophilic tubulointerstitial infiltration is seen in 75% of patients. ¹⁰ Interestingly, renal involvement may vary from “minimal change glomerulonephritis” to “membranous glomerulonephritis.” Overall, however, membranous glomerulonephritis is the most commonly observed lesion.

Steroid therapy is usually effective in most patients with KD. However, recurrence rates as high as 66.6% have been reported following steroid tapering. Atmadja et al. have recently reported success with cyclosporine therapy in steroid-resistant patients. ¹¹ Surgical intervention is another alternative. Surgery is associated with only a 6.6% risk of recurrence of the disease. ¹² Renal transplantation may be necessary in some patients with chronic renal failure.

As is obvious from the above discussion, KD is a rare cause of lymphadenopathy. It should be considered in all patients presenting with lymphadenopathy and nephrotic syndrome.