Risk of Nephritis and Recurrence in Kimura Disease: A Retrospective Study

Abstract

Background: Kimura disease (KD) presents a diagnostic challenge to clinicians because of its rarity and atypical symptoms in its early stages, and it is difficult to treat and prone to recurrence or involvement of other organs.

Aims and objectives: This study aims to investigate the possible relevance of renal involvement and recurrence by analysing the clinical presentations, laboratory results, histopathological features, therapeutic data and follow-up results of KD.

Materials and methods: A total of 27 patients diagnosed as KD in two hospitals from January 1999 to December 2021 were analysed retrospectively in this study based on the diagnosis of histopathology.

Results: KD mainly affected male more than female (8:1) with the onset age ranging from 3 to 58 years (median 29.8 years). The common initial symptoms included subcutaneous soft tissue or lymph node enlargement, non-specific skin lesions and proteinuria. One patient presented cough and expectoration as the first symptoms. KD patients often had high levels of serum immunoglobulin E (IgE) and basophils, which exhibited a significantly positive correlation with renal involvement and recurrence (p < 0.05). Early mass resection could prevent the development of nephritis and decrease the risk of relapse (p < 0.05).

Conclusion: KD should be noted in patients presenting with intractable and relapsing atopic skin lesions and (or) subcutaneous mass. Patients with high levels of serum IgE and blood basophils may be prone to developing KD-associated nephritis and predict a high risk of recurrence. Early surgical removal of the mass may result in a better prognosis.

Keywords: Basophils; Kimura disease; nephritis; recurrence; serum IgE.

Figure 1

The patients present non-specific lesions and masses. (a) Purpuric erythematous papules on extremities; (b) Multiple nodules in left cheek; (c) Itching erythema on elbow; (d) Single nodule in head with scale and telangiectasia; (e) A soft tissue enlargement in deep elbow detected by colour doppler ultrasound

Figure 2

The scatter plot graph and correlated factors to nephritis and recurrence analyzed by Spearman’s rank test

Figure 3

The histopathology of skin lesion indicated hyperplasia of lymphocytes and lymphoid follicles in dermis, extensive eosinophil infiltration, vascular and endothelial proliferation with pericapsular and capsular fibrosis (a HE ×40, b HE ×400). The histopathology of soft tissue indicated lymphatic follicular hyperplasia with interstitial small vessel hyperplasia, extensive eosinophilic infiltration and occasional eosinophilic abscesses (c HE ×40, d HE ×400)

Figure 4

The histopathology of renal biopsy. (a) The glomerular mesangial area is broadened and the basement membrane-like substance is increased. (b) The glomerular podocyte foot processes are widely fused (>50%), more microvilli, and the expanded endoplasmic reticulum and vacuoles are seen in the cytoplasm