scholarly journals Efficacy of rituximab in thymoma associated minimal change disease: case report

2021 ◽  
Vol 22 (1) ◽  
Author(s):  
Zhour El Ouafi ◽  
Clovis Mugnier ◽  
Robin Jeannet ◽  
Clément Danthu ◽  
Marion Duval ◽  
...  

Abstract Background Thymomas have been associated with a broad spectrum of autoimmune diseases. Minimal change disease (MCD) is the most frequent pathological lesion reported. Pathophysiological mechanisms involved in secondary MCD, and linking MCD to thymoma are not yet fully explained, although the hypothesis of T cell dysfunction has been suggested. The fundamental therapeutic principles are steroids and surgical treatment of thymoma, but failures and relapses often require immunosuppressant combinations. Case presentation A 62-year-old female was admitted in our unit for a nephrotic syndrome associated with a thymoma. The diagnosis of thymoma associated MCD was confirmed by kidney biopsy. After surgical resection of the thymoma and steroid therapy, no remission was observed. Immunosuppressive therapy was then intensified with introduction of rituximab. Here, we report a steroid-resistant nephrotic syndrome secondary to MCD associated thymoma, which achieved complete remission after rituximab therapy. To the best of our knowledge, this is the first report of the use and efficacy of rituximab therapy in this pathology. Conclusions Our case report suggests that primary and secondary MCD may share similar pathophysiological mechanisms. It does not allow us to draw any conclusions about the mechanism of action of rituximab, but we believe this report argues for the safety and efficacy of rituximab use in thymoma-associated MCD, and therefore constitutes a rationale for future studies.

2021 ◽  
Vol 6 (2) ◽  
pp. p10
Author(s):  
Christina Danai Skondra ◽  
Christalleni Christodoulidou ◽  
Theodoros Tegos ◽  
Christina Vourlakou ◽  
Aggeliki Paikopoulou ◽  
...  

We report the case of a 64 year old male patient with a recent diagnose of pulmonary nodules with pleural thickening who was admitted to the nephrology department of our hospital for further evaluation of nephrotic syndrome (NS). Malignant epithelioid mesothelioma (MEM) was diagnosed after pleural biopsy while the renal biopsy showed minimal change disease (MCD) with acute tubular necrosis and interstitial lymphoplasmacytic infiltrate. MCD is the most common cause of nephrotic syndrome in children while it accounts for a smaller proportion of NS in adults. The underlying cause of MCD is unclear (Cameron, 1987). Evidence so far suggests that T-cell dysfunction results in the production of a circulating factor directly affecting the glomerular capillary wall with consequent foot process fusion. MCD as paraneoplastic syndrome is common in hematological disorder while the incidence in lung pulmonary malignancies is rare. Early recognition leads to an early diagnosis of malignancy improving the outcome and diminishing the associated co-morbidities.


Author(s):  
Vânia Junqueira ◽  
Beatriz Donato ◽  
Catarina Teixeira ◽  
Maria Isabel Mascarenhas ◽  
Isabel Costa Silva ◽  
...  

ABSTRACT Minimal change disease accounts for up to 25% of the cases of nephrotic syndrome in adult population. The allergic mechanism has been associated with minimal change disease and allergens have been implied, namely insect stings. We present a case report of a woman with new onset of nephrotic syndrome after a non-hymenoptera insect sting, with biopsy-proven minimal change disease, that was accompanied by a pulmonary thromboembolism process. Complete remission with glucocorticoid therapy was observed, with sustained response for 6 months after discontinuation. A new exposure to insect sting in the same geographical region and season triggered a nephrotic syndrome relapse. Subsequent avoidance of the place resulted in a sustained remission for more than 4 years.


2017 ◽  
Vol 62 (2) ◽  
pp. 54-57 ◽  
Author(s):  
Wen-Fang Chiang ◽  
Jenq-Shyong Chan ◽  
Pauling Chu ◽  
Chia-Chao Wu

Introduction Pulmonary embolism is a potentially life-threatening complication of nephrotic syndrome. Syncope is rarely reported as an initial presentation of pulmonary embolism in nephrotic patients. Case presentation We describe a 35-year-old man who was taking steroids and diuretics for relapse of minimal change disease who presented after a syncopal event. The patient was hypotensive and had distended neck veins. The major laboratory findings were hypoalbuminemia with mild proteinuria. The findings on electrocardiography, chest radiography, and echocardiography and the elevated plasma D-dimer level raised suspicion of pulmonary embolism. Thrombi in the bilateral main pulmonary arteries on chest computed tomography together with compromised hemodynamics were consistent with the diagnosis of massive pulmonary embolism. He received anticoagulant treatment and the disease resolved. Conclusion Pulmonary embolism should be considered as a cause of syncope in patients with nephrotic syndrome, despite the absence of severe hypoalbuminemia and proteinuria, especially in patients taking concurrent steroid and diuretic therapy.


2014 ◽  
Vol 2014 ◽  
pp. 1-8 ◽  
Author(s):  
Susan Swee-Shan Hue ◽  
Sufi Muhammad Suhail ◽  
Jason Chon Jun Choo ◽  
Nurhashikin Yusof ◽  
Alwin Hwai-Liang Loh ◽  
...  

Minimal change disease constitutes a major cause of nephrotic syndrome. It is regarded as a non-immune-complex mediated primary glomerulopathy and pathogenetically is characterised by podocyte injury and effacement of foot processes; therefore, it is also classified as a type of podocytopathy. T cell dysfunction with increased levels of a soluble glomerular permeability factor has been proposed to play a major role in the pathogenesis of minimal change disease. It has been therefore suggested that a dysfunction of regulatory T cells, the orchestrators of immune homeostasis, could be implicated in perpetuating T cell activation in this condition. However, the actual contribution of regulatory T cell dysfunction in the immunopathogenesis of primary minimal change disease is still largely unclear. We here propose a theoretical model based on the available evidence.


Nephrology ◽  
2010 ◽  
Vol 15 (5) ◽  
pp. 592-592 ◽  
Author(s):  
Ying Zhou ◽  
Huaizhou You ◽  
Jing Xin ◽  
Yong Gu ◽  
Shanyan Lin ◽  
...  

2019 ◽  
Vol 2019 ◽  
pp. 1-3 ◽  
Author(s):  
Janet K. Lee ◽  
Kathleen Murray ◽  
Swetha Renati

Nephrotic syndrome is defined by three characteristic features including proteinuria of >3 g in 24 hours, hypoalbuminemia of less than 3 g/dL, and peripheral edema. Multiple nephropathies can result in nephrotic syndrome. Most commonly, minimal change disease is seen in children under the age of 10, while adults are more commonly found to have membranous nephropathy. Hypercoagulability and thrombotic sequela can be seen in nephrotic syndrome, regardless of underlying etiology, and thrombosis is most commonly seen in deep veins of the lower extremities and renal veins. Our case identifies an adult with previously diagnosed and treated for minimal change disease who presented with weight gain, peripheral edema, foamy urine, headache but no neurologic deficits. The patient was found to have near to complete occlusion of the entire superior sagittal sinus, near complete occlusion of the left transverse and sigmoid sinuses, and nonocclusive thrombus in the right sigmoid sinus. She was treated with heparin and IV steroids then transitioned to warfarin and PO steroids, respectively, with resolution of symptoms. This case report emphasizes on the importance of recognizing CVST as a potential complication of nephrotic syndrome at both initial presentation and relapse.


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