Kimura disease with renal impairment: case series and literature review / 中华肾脏病杂志

Objective:To analyze the clinical and pathological characteristics, treatment and prognosis of renal changes in patients with Kimura disease and improve the clinicians′ understanding on renal manifestations of Kimura disease.Methods:The clinical data of Kimura disease patients with definite diagnosis and detailed data in Peking Union Medical College Hospital from January 1980 to August 2020 were retrospectively analyzed. The patients were divided into renal impairment group and non-renal impairment group according to whether the kidney was involved or not and the related clinical data between the two groups were compared. The patients presenting with nephrotic syndrome were followed up.Results:There were 60 patients with Kimura disease confirmed by pathological diagnosis with 48 males. The median age was 33(3, 62) years old, and the median duration was 36(12, 111) months. There were 18 cases complicated with renal injury in 49 patients with complete routine urine and renal function examination and the main manifestations of renal injury were proteinuria and/or microscopic hematuria. There was no significant difference at age, sex and absolute value of eosinophils between the two groups (all P>0.05). Compared with the renal inpairment group, patients in non-renal inpairment group had longer course of disease, higher levels of hypersensitive C-reactive protein and erythrocyte sedimentation rate, and lower median values of total eosinophils and total IgE, but there was no statistically significant difference (all P>0.05). Among the patients with renal involvement, 6 patients met the diagnostic criteria for nephrotic syndrome, and 5 of them completed renal biopsies. The renal pathological diagnosis was membranous nephropathy in 2 cases and minimal change disease in 3 cases, and no interstitial eosinophil infiltration was found in renal biopsy tissues. These patients had a good response to glucocorticoids and/or immunosuppressive therapy, and achieved complete remission of nephrotic syndrome; at the same time, lymphadenopathy caused by Kimura disease could be well controlled. Conclusions:Kimura disease can combine with various renal lesions, and the pathology of nephrotic syndrome can be membranous nephropathy or minimal change nephropathy. After energetic treatment of glucocorticoids and/or immunosuppressive therapy, nephrotic syndrome can be completely relieved, and lymphadenopathy can be well controlled. The relationship between Kimura disease and renal disease needs further study.

Kimura Disease as a Rare Cause of Proptosis: A Case Report

ABSTRACT@#Kimura disease (KD) is a rare chronic inflammatory disorder of unknown aetiology that primarily affects the head and neck region with lymph node involvement. Young to middle-aged adult Asian males are predominantly affected. The most common presentation is painless subcutaneous swelling in the head and neck region, while proptosis or orbital involvement is very rarely reported. KD shares some features with other inflammatory and neoplastic disorders, including lymphoma; thus, investigations to confirm the diagnosis should not be delayed. Systemic corticosteroids are commonly used to treat KD and show an excellent response; however, the optimal treatment is still uncertain, and KD has a high recurrence rate. We describe the case of a patient with KD who presented with proptosis and post-auricular swelling, which responded well to oral prednisolone treatment.

A rare case of Kimura lymphadenpathy in elderly female

Kimura's disease (KD) is a chronic inflammatory disease, a rare variety. It usually presents as non-tender subcutaneous swelling in head and neck region, predominantly in preauricular and submandibular area and is often associated with cervical lymphadenopathy, marked peripheral eosinophilia and an elevated immunoglobulin E (IgE) level. Renal involvement is the only systemic manifestation.4 Nephrotic syndrome is the most common manifestation of renal disease in KD. Diagnosis through Fine needle aspiration cytology (FNAC) is misleading and can easily be mistaken as a malignant disease. So, diagnosis is therefore only established by histopathological examination. The treatment of KD involves one of three major approaches are surgical excision, irradiation, or steroid therapy. Surgical excision is recommended as the treatment of choice but carries recurrence rates of 33-50%. In our study, we are reporting a case of elderly female who presented with left sided cervical lymphadenopathy for which excision biopsy was done and the histopathology examination shown as kimura lymphadenopathy, a rare case.

Kimura's disease involving bilateral lacrimal glands and extraocular muscles along with ipsilateral face: A unique case report

A 23 year female presented with bilateral recurrent swelling of eyelids along with ptosis and proptosis for last 3 years. She also had swellings over the right cheek, parotid gland, and retro auricular area along with regional lymphadenopathy. Systemic laboratory workup revealed raised serum IgE and a high peripheral eosinophil count. Computed tomography and magnetic resonance imaging showed bilateral enlargement of extraocular muscles, lacrimal glands, and ipsilateral parotid gland. Excision biopsy of the retro-auricular lymph node was suggestive of Kimura's disease (KD). The patient responded well to systemic corticosteroid. KD rarely affects orbit, but it should be included in the differential diagnosis of inflammatory diseases of the orbit. To our knowledge, this is the first reported case of KD from India involving the orbit, lacrimal gland, extraocular muscles, parotid gland and buccal area.

Kimura disease with nephrotic syndrome in a child- A rare association

Lymphadenopathy along with various systemic manifestations is commonly encountered in pediatric patients, tuberculosis being the commonest etiology. Occasional patients may present a diagnostic conundrum. Here, the authors report an unusual manifestation of Kimura disease (KD) presenting as nephrotic syndrome associated with mesenteric lymphadenitis in an 11-year-old male child. KD is a chronic inflammatory disorder of unknown etiology. It typically affects young adult males in the age range of 27-40 years and usually presents as painless itchy nodular masses in the head and neck region. The involvement of mesenteric lymph nodes along with a very young age of presentation makes it a rare case, posing a diagnostic challenge for the unsuspecting physician.

Bilateral Involvement of Juvenile Temporal Arteritis Associated with Kimura Disease

Juvenile temporal arteritis (JTA) is a localized nodular arteritis confined to the temporal artery without evidence of systemic inflammation, and it occurs mainly in patients younger than 50 years. From the first case report, the pathological features of JTA have been suspected to be the morphological equivalent of Kimura disease (KD), which has been supported further by the concurrent cases of JTA with KD. We present the first case of bilateral JTA accompanying KD, which was confirmed by histological and ultrasound evaluations and supports the hypothesis that JTA is a manifestation of KD. The un-excised JTA lesion was resolved completely after corticosteroid therapy with no recurrence.

Kimura Disease: A differential diagnosis in a nephrotic child

@#Kimura disease presents as benign lesion and is commonly present among the Asian population. It is a disease with a favourable prognosis and a peak age of onset in the third decade. It is a chronic inflammatory disorder of unknown etiology that involves the lymph nodes and subcutaneous tissues of the head and neck region. We report a case of a 15-year-old boy with multiple Kimura lymphadenopathies involving the left posterior auricular region as well as the anterior and posterior triangles of the neck.

Anaplastic large cell lymphoma with marked peripheral eosinophilia misdiagnosed as Kimura disease

Hypereosinophilia, defined as an absolute eosinophil count of >1,500/μL, can be caused by a number of allergic, infectious, paraneoplastic and neoplastic disorders. In cases of hypereosinophilia with lymphoid proliferation, pathological confirmation is essential to exclude either myeloid or lymphoid malignancy. A 38-year-old woman with both cervical lymphadenopathies and peripheral blood eosinophilia visited our clinic. She had already performed core biopsy of lymph nodes and diagnosed as Kimura disease at a regional hospital. At the time of our clinic visit, there were no palpable cervical lymph nodes. The blood test showed hypereosinophilia with a high total IgE level. There was no evidence of tissue infiltration of eosinophils except for duodenitis with eosinophilic infiltration. Based on these findings, she was diagnosed as Kimura disease. She treated with high-dose systemic corticosteroid (1 mg/kg) and additional immunosuppressants sequentially used cyclophosphamide and cyclosporine. However, her eosinophilia waxed and waned, and a left inguinal mass was newly found. Excisional biopsy findings showed large atypical lymphoid cells with numerous eosinophilis, and immunohistochemistry showed CD3+, CD20−, CD30+ and anaplastic lymphoma kinase (ALK). The final diagnosis was ALK-negative anaplastic large cell lymphoma. We report a case of anaplastic large cell lymphoma with marked peripheral eosinophilia misdiagnosed as Kimura disease. In the case of hypereosinophilia with lymphadenopathy, it is necessary to differentiate hematologic diseases through immunochemical staining.

Clinicopathological analysis of 22 cases of cutaneous epithelioid hemangioma / 中华皮肤科杂志

Objective To investigate clinical and histopathological features of cutaneous epithelioid hemangioma (EH).Methods Clinical and histological data were collected from 22 patients with EH and analyzed.Results Of the 22 EH patients,10 were male,and 12 were female.The age at onset ranged from 16 to 62 years,with an average age of 45.91 years.The duration of disease varied from 1 month to 20 years,with an average disease duration of 37 months.Skin lesions most frequently occurred on the scalp in 14 cases (63.6％),followed by the ear in 6 cases (27.3％),the forehead in 2 cases (9.1％),and the thigh in 1 case (4.5％).Lesions affected both the scalp and forehead in 1 case.Histopathological examination of the 22 cases revealed vascular proliferation.The blood vessels were lined with epithelioid endothelial cells,and a large number of lymphocytes and eosinophils infiltrated around the vessels.The dermis were involved in all cases,and subcutaneous tissues were involved in 8 cases (36.4％).Immunohistochemical examination of 6 cases showed positive staining for CD31,CD34 and factor Ⅷ in blood vessel walls.Conclusion EH is an uncommon disease characterized by vascular proliferation and inflammatory infiltration,and clinical manifestations in combination with histopathological examination facilitate its diagnosis.

Basophils are increased and express increased levels of interleukin-4 in the parotid lesions of Kimura disease

BACKGROUND: Kimura disease (KD) is a systemic soft-tissue disease that leads to formation of painless masses in lymph nodes, with the highest predilection for the head and neck and especially the parotid gland. KD lesions are characterized by marked eosinophil infiltration, production of IgE and increased expression of T-helper type 2 (Th2) cytokines (interleukin [IL]-4, IL-5, etc.). Skewing to a Th2 inflammation is also demonstrated in the peripheral blood, with elevated eosinophils and high IgE levels. It is thought that basophils may play important roles in orchestrating this Th2 inflammation via IL-4 production leading to the induction of IgE synthesis as well as eosinophil infiltration. However, there are no reports as yet on the role of basophils in KD. OBJECTIVE: The present study was performed to investigate the potential role of basophils in the pathogenesis of KD. In this context we also examined the expression of IL-4 in basophils in the KD lesions. METHODS: By immunohistochemistry using a monoclonal antibody against a basophil marker ProMBP1 we investigated the number and distribution of basophils in the KD lesions. By double immunohistochemistry we analyzed the colocalization of IL-4 in basophils. RESULTS: There was an increased number of basophils infiltrating the KD parotid gland lesions as compared to that in normal control parotid tissue. By double-immunofluorescence we found that approximately 7% of IL-4-positive cells in KD patients' parotid glands were basophils. CONCLUSION: Basophils may also play a role in the pathogenesis of KD, leading to the induction of IgE synthesis and eosinophil infiltration.

CT and MRI manifestations of Kimura disease in head and neck / 中国医学影像技术

Objective To explore the CT and MRI manifestations of Kimura disease in head and neck.Methods The CT and MR findings of 20 cases with Kimura disease confirmed by pathology were collected.The lesion location,boundary,size,number,density of CT and signal intensity of MRI were analyzed.Results In 20 cases,multiple lesions were seen in 19 cases.There were 11 cases involving parotid gland,6 cases involving the submandibular area,2 cases in cheek,1 case in palate,2 cases involving the upper arm,1 case involving axillary fossa,1 case involving cervical lymph node alone,and 8 cases with subcutaneous nodules in the front or back of the ear,16 cases accompanied by lymphadenopathy in the neck (8 cases with lymphadenopathy in the parotid gland).The largest diameter of lesion was (3.09± 1.08)cm.The margin was blurred in 19 cases and well defined in 1 case.The isodensity lesions were found in 16 cases and slightly lower density lesions were in 4 cases in non-contrast enhanced CT scan.Calcification was not found in all cases.Homogeneous enhancement was found in 16 cases and inhomogeneous enhancement was found in 1 case.The enhancement degree varies from mild to strong enhancement.Homogenous signal and strong enhancement was found in 2 cases with MR scan.Conclusion The typical manifestations of Kimura disease are involvement of parotid gland and subcutaneous fat,accompanied by lymphadenopathy in the neck,homogeneous density on CT or MRI,which may indicate Kimura disease.

Imaging characteristic of Kimura disease and literature review / 实用放射学杂志

Objective To explore the CT and MRI findings of Kimura disease in different location.Methods The CT and MRI obtained in 19 patients with histologically proved Kimura disease were reviewed retrospectively.Results Of these 19 patients with Kimura disease, the head and neck was involved in 16 patients, other locations less commonly involved were the lung(n=1),chest wall(n=1),liver and spleen(n=1).Kimura disease of head and neck,the density abnormal appeared as nodular,mass or diffuse on CT imaging, the CT value of lesion was similar to muscle, MRI manifested as a slightly hypo-or isointense lesion on T1WI and hyperintense on T2WI,most of the lesions demonstrated moderate or marked enhancement on postcontrast CT and MR images.Kimura disease of lung and chest wall,lesion appeared as a solid mass on imaging, there was moderate enhancement in the lung lesion and rim enhancement in the chest wall lesion.Kimura disease of liver and spleen: lesion showed nodular, mild and gradual upward enhancement.Conclusion Imaging findings of Kimura disease are not characteristic, it is very essential to know the lesion combined with the laboratory and pathologic features.

The clinical feature, pathology, and prognosis of Kimura disease complicated with nephrotic syndrome in children / 临床儿科杂志

Objective To explore the clinical feature of Kimura disease complicated with nephrotic syndrome in children. Methods The clinical data from 4 children with Kimura disease complicated with nephrotic syndrome were retrospectively analyzed. Results In all of the 4 male children, level of serum IgE was increased but level of eosinophils was not increased in peripheral blood. The renal pathological manifestations were different among them. Two cases had acute tubular injury, one case had pathological changes in repeated renal biopsy, and one case had a little of eosinophils infiltration in renal interstitium. All of them were sensitive to oral hormone treatment. Except that one case had no relapse yet in short follow-up period, the other 3 cases had relapsed and all manifested as positive urinary protein without lymphadenectasis. The 3 cases with recurrence were treated by combined immunosuppressive agents, the palindromia of two cases were reduced which were combined with tacrolimus. Conclusion The Kimura disease complicated with nephrotic syndrome in children has a long course and different renal pathological manifestations. It is sensitive to hormone treatment but easy to relapse, and the treatment should be combined with immunosuppressive therapy. Tacrolimus may have the effect on reducing relapse and delaying the progress of renal pathology.

Bilateral extraocular muscles enlargement from Kimura’s disease of the orbit.

Kimura’s disease (KD) is a rare chronic inflammatory disease of unclear etiology, characterized by subcutaneous nodules, mainly in the head and neck region, frequently associated with regional lymphadenopathy. Orbital involvement is infrequent and when it occurs, usually affects the eyelid or the lacrimal gland. We report a case of a 44-year-old man that presented with bilateral slowly progressive proptosis that was initially misdiagnosed as Graves’ Ophthalmopathy. 15 months of worsening proptosis and the development of facial and temporal swelling led to further investigation. Computed tomography and magnetic resonance imaging showed enlargement of all recti muscles and diffuse orbital infiltration. An orbital biopsy was performed and was consistent with the diagnosis of KD. Long term oral corticosteroid showed marked improvement of proptosis and facial swelling. This case serves to emphasize that KD should be included in the differential diagnosis of inflammatory diseases of the orbit, even when characterized by predominant involvement of the extraocular muscles.

Kimura’s Disease of Face: A Clinicopathological Presentation and Review.

Kimura’s disease is an extremely unusual disorder of benign nature affecting the connective tissue, and primarily seen in young males in countries of Asian pacifi c region particularly in China and Japan. This is characterized by three essential components of diagnostic importance. (a) Painless benign subcutaneous masses and rarely lymph nodes most predominantly in the head and neck region and usually solitary, (b) Gross eosinophilia in the blood and also in the affected tissue, (c) Markedly elevated serum immunoglobulin E levels. We are reporting this extensive case in a male aged 28 years, and the lesion started when he was 15 years old, affecting the cheeks and submental region, right parotid gland, and associated lymph nodes. Since we operated all the three masses, we present our operative and clinical experiences along with histopathological fi ndings. We are also reviewing the cases already reported earlier and the problems of clinical diagnosis and to differentiate from other diseases which may simulate this disease. Relative merits of all the treatment protocols and the outcome of the disease is also discussed in the light of our experience with this patient.

Kimura’s Disease: A Rare Cause of Chronic Lymphadenopathy in a Child

Kimura’s disease is an uncommon entity that affects adults, with a predilection for the Asian population. This may rarely be encountered in children, and the knowledge of this fact is essential to rule out the remote possibility of Kimura’s disease in children with a slow-growing painless mass in the head and neck region. In this case report, we document this disease in an 8-year-old boy with a slow-growing swelling in the right posterior auricular region.

Kimura’s Disease: Diagnostic Challenge and Treatment Modalities

Case Report: Five cases of Kimura’s disease had been treated in our centre from year 2003 to 2010. All cases were presented with head and neck mass with cervical lymphadenopathy. Surgical excision was performed for all cases. Definite diagnosis was made by histopathological examination of the resected specimens. One out of five cases developed tumour recurrence four years after resection. Conclusion: Surgical excision is our choice of treatment because the outcome is immediate and definite tissue diagnosis is feasible after resection. Oral corticosteroid could be considered as an option in advanced disease. However, tumour recurrence is common after cessation of steroid therapy.

Remission of secondary membranous nephropathy in a patient with Kimura disease after surgical resection

Kimura disease (KD) is an eosinophilic, granulomatous, benign, chronic inflammatory disease with an unknown etiology. A 33-year-old woman visited our hospital because of a palpable, left subclavian mass, a left scapulo-anterior pseudoaneurysm, and nephrotic syndrome. Her subclavian lymph node biopsy examination result was consistent with KD, and results of a renal biopsy indicated secondary membranous nephropathy. After renal histological examination confirmed nephropathy, treatment with prednisolone and cyclosporine was initiated, which was maintained for over 1 year. However, this therapy only provided a transient improvement in proteinuria. One year after commencing the treatment, both proteinuria and azotemia aggravated as the left axillary mass doubled in size. Finally, the mass was surgically excised, following which the azotemia rapidly normalized and proteinuria resolved within 1 month. This case shows that tumor resection in a patient with KD with secondary nephropathy may resolve secondary renal manifestations. Furthermore, reversible renal dysfunction may be caused by unknown secreted molecules.

An Unusual Clinical Presentation of Kimura's Disease Occurring in the Mesenteric Lymph Nodes.

Kimura's disease is a rare inflammatory disorder of unknown aetiology primarily seen in young Asian males. The disease is characterized by a triad of painless subcutaneous masses in the head and neck region, blood and tissue eosinophilia, and markedly elevated serum immunoglobulin E (Ig E) levels. Clinically the subcutaneous nodules occur predominantly in the head and neck region of young males. However, we report the case of a 60 year old male presenting with mesenteric lymphadenopathy diagnosed with Kimura's disease.