scholarly journals Rituximab Treatment in a Patient with Kimura Disease and Membranous Nephropathy: Case Report

2021 ◽  
pp. 116-123
Author(s):  
Roald Vissing-Uhre ◽  
Alastair Hansen ◽  
Susanne Frevert ◽  
Ditte Hansen

Kimura disease (KD) is a chronic, inflammatory disorder with slowly developing subcutaneous tumor-like swellings, often occurring in the head and neck region. KD is diagnosed based on histology, elevated levels of immunoglobulin type E, and increased peripheral eosinophil granulocytes. KD may coexist with glomerular renal diseases, and this case report is based on a patient with KD-associated membranous nephropathy. Patients with membranous nephropathy without KD have demonstrated responsiveness to treatment with monoclonal anti-CD20 antibodies. This case report is the first to investigate the effect of rituximab treatment in a patient with KD-associated membranous nephropathy. A 30-year-old Italian man living in Denmark was diagnosed with Kimura’s disease based on subcutaneous nodules with eosinophil angiolymphoid hyperplasia. The patient was admitted to the hospital due to nephrotic syndrome. Serology showed eosinophil granulocytosis and negative PLA2-receptor antibody. Renal biopsy showed membranous nephropathy, and the patient was treated with systemic methylprednisolone followed by cyclosporin and then cyclophosphamide with only partial remission. Ultimately, treatment with intravenous rituximab was initiated, which resulted in overall remission and no nephrotic relapses at 30 months of follow-up. Thus, intravenous rituximab effectively decreased proteinuria and prevented nephrotic relapses in a patient with treatment-refractory membranous nephropathy due to KD.

Author(s):  
Vinay Bharat ◽  
Abhishek Gupta ◽  
Rani Bansal ◽  
Priya Gupta ◽  
Mamta Gupta

Kimura’s disease is a rare chronic inflammatory disorder present in 2nd and 3rd decade. It has a predilection for head and neck region presenting as a slowly growing painless swelling. It is usually accompanied by peripheral eosinophilia and elevated serum IgE and hence it was initially thought to be of allergic origin. Histologically the lesions are characterized by reactive hyperplasia of lymph nodes, eosinophilic infiltration and increase in postcapillary venules. Authors have reported a male patient with a slowly growing right sided neck swelling which is recurring even after course of steroids and excision done twice at an interval of 6 months. Kimura’s disease although a benign Lymphoid disorder but the incidence of recurrence despite taking treatment is a cause of much concern for the patient.


2021 ◽  
Vol 14 (1) ◽  
pp. e232627
Author(s):  
Jacqueline Ho ◽  
Sophie Walter ◽  
Richard J Harvey

Kimura’s disease is a rare, benign, chronic inflammatory disorder characterised by its eosinophilic infiltrate. Patients often present with one or more progressively enlarging subcutaneous lymph nodes in the head and neck region or enlarging salivary glands. We describe the case of a 26-year-old man presenting with severe peripheral eosinophilia and upper airway inflammatory symptoms, who later developed cervical lymphadenopathy and formally diagnosed with Kimura’s disease. Based on our English-language MEDLINE literature search, to our knowledge this is the first case report describing treatment of Kimura’s disease with mepolizumab.


2017 ◽  
Vol 25 (1) ◽  
pp. 59-61 ◽  
Author(s):  
Suman Kumar Das ◽  
Mukesh Kumar Singh ◽  
Indranath Kundu ◽  
Swapan Kumar Ghosh

Introduction Kimura’s Disease is a chronic inflammatory disorder of lymph node which is very rare in Indian population. Case Report A 15 year old boy with multiple postauricular swelling for 18 months presenting in OPD and diagnosed having eosinophilia. Then excision biopsy was taken, which indicates Kimura’s Disease. Patient was treated with high dose of corticosteroid. Conclusion Kimura’s disease, though rare should be kept in mind for treating a patient with lymphadenopathy with eosinophilia or high IgE level, because it can spare the patient unnecessary invasive procedure.


2022 ◽  
Vol 4 (1) ◽  
pp. 01-03
Author(s):  
Hafeza MS ◽  
Vanitha Palanisamy

Here we present a 43-years old Malay man with Kimura’s disease of the right parotid gland. Asian men have a predisposition for this chronic inflammatory disorder of unknown etiology mostly presenting at the head and neck region. The management in dealing with this patient is not straight forward as the presentation mimics a subcutaneous tumor-like mass in which main differential diagnosis includes lymphoproliferative disorders and parotid neoplasm. Moreover, cytological investigation is inadequate, and this necessitates the use intra-operative frozen section prior to tumor debulking. Without proper investigation, misdiagnosis can occur and potentially expose the patient to unnecessary extensive surgical procedures. Here we discuss step by step approach in managing this patient.


2019 ◽  
Vol 101 (5) ◽  
pp. 309-312
Author(s):  
C Samian ◽  
S Ghaffar ◽  
V Nandapalan ◽  
S Santosh

Introduction Malakoplakia is a rare chronic inflammatory disorder, predominantly affecting the urinary tract. In the head and neck region, it is very rare and may confuse the clinicians during investigations, as features may mimic malignancy. Materials and methods We report a case of malakoplakia involving the parotid gland and review of the reported cases of malakoplakia in head and neck region. Results Histologically, this is the first classic case report of malakoplakia involving the parotid gland in the world literature. A total of 49 cases have been reported in the head and neck region; 38.7% of these are cutaneous. In soft tissue, the tongue is the most common site. Salivary gland involvement is very rare. Previously, submandibular salivary gland involvement has been reported. Conclusion A possibility of malakoplakia should be considered as a differential diagnosis in patients with enlarged head and neck masses. Histology is essential to diagnose this benign inflammatory disorder and to differentiate from a malignant process.


2021 ◽  
Vol 16 (2) ◽  
pp. 259-265
Author(s):  
V Sha Kri Eh Dam ◽  
Irfan Mohamad ◽  
Evelyn Li Min Tai ◽  
Adil Hussein ◽  
Khairil Amir Sayuti ◽  
...  

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.


Author(s):  
Firamir Bin Zulkifli ◽  
Mawaddah Binti Azman ◽  
Loong Siow Ping

Kimura disease is an idiopathic chronic inflammatory disorder involving the subcutaneous tissue. It favors the head and neck region, although presentation over atypical sites has been reported. This disease occurs spontaneously, has a characteristic indolent growth pattern but has a high tendency to recur locally after treatment. Due to its benign nature, there is still no clear consensus on the optimal management of this rare disorder. Various treatment options are available which include systemic steroids, antihistamines, immunosuppressant, chemotherapy, radiotherapy, and surgical excision. Presently, the disease remains a therapeutic enigma as the pathogenesis is still a mystery and its tendency to recur following treatment proves to be discouraging. Herein we report a rare case of Kimura disease involving the supra-auricular region who underwent surgical excision.


Author(s):  
Harshi Dhingra ◽  
Ruchi Nagpal ◽  
Asif Baliyan ◽  
Sathyavathi R Alva

Kimura disease is a rare chronic inflammatory disorder of unknown cause, primarily seen in young Asian males. The disease is characterized by painless subcutaneous swelling, blood and tissue eosinophilia and raised IgE levels. Early diagnosis of Kimura’s disease may spare the patient from unnecessary invasive diagnostic procedure. We describe a case of Kimura disease in a 14-year old male presenting with left submandibular swelling and also provide a brief review of the literature. 


2010 ◽  
Vol 2010 ◽  
pp. 1-4 ◽  
Author(s):  
Mohamed Ashraf Fouda ◽  
Osama Gheith ◽  
Ayman Refaie ◽  
Mohamed El-Saeed ◽  
Adel Bakr ◽  
...  

Kimura disease (KD) is a chronic inflammatory disorder with angiolymphatic proliferation, usually affecting young men of Asian race but is rare in other races. The etiology of KD is still unknown. It is often accompanied by nephrotic syndrome. Herein, we present an atypical manifestation of Kimura disease occurring in a Caucasian man with steroid-responsive early membranous glomerulonephritis. Kimura disease can present atypically in a middle-aged Caucasian man with secondary steroid-responsive nephrotic syndrome. Steroid, endoxan, and MMF can be used safely and successfully in such situation. The diagnosis of KD can be difficult and misleading, and patients with this disease are often evaluated using avoidable procedures by just not being aware of KD.


2021 ◽  
Vol 6 (2) ◽  
pp. 61-64
Author(s):  
Zalilah Musa ◽  
Abdul Razak Ismail ◽  
Irfan Mohamad

Introduction: Kimura disease is a rare chronic inflammatory disorder affecting the subcutaneous tissue. It is of unknown etiology mainly occurring in head and neck region. Case report: A 34-year-old Malay lady presented with a gradually enlarging, painless right nasal bridge mass for one year duration. Clinical examination revealed a firm, immobile swelling measuring 2 cm x 2 cm. Nasal endoscopy showed no extension of the mass into the right nasal cavity. Surgical excision was performed where histopathological examination reported as finding consistent with Kimura disease. Conclusion: Kimura disease is extremely a rare benign disease, which can affect the head and neck structures. Manifestation at the nasal region is an extremely rare occurrence. Histopathological examination is needed to diagnose the disease.


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