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Membranous glomerulonephritis is the most common glomerular disease in adults. Its primary form has been characterized with formation of phospholipase A2 receptor antibodies. Malignancy, infections, and autoimmune disorders are the most common causes of secondary membranous glomerulonephritis. We present a case of a 55-year-old African American fem...
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![Figure 4: Pathophysiology of thyroid eye disease (TED)[11].](profile/Ashraf-Shoma/publication/359695372/figure/fig2/AS:11431281104594269@1670148016179/Pathophysiology-of-thyroid-eye-disease-TED11_Q320.jpg)
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Citations
... [1,2] Nephrotic syndrome is rarely reported in medical literature in association with SMA. [3] Here, we report a case of a middle-aged gentleman presenting with SMA and primary membranous nephropathy (PMN) and highlight the treatment dilemmas associated with the management of nephrotic syndrome in the background of significant muscle dysfunction. ...
... [2] Membranous nephropathy has been associated with neurological disorders like myasthenia gravis and chronic inflammatory demyelinating polyneuropathy. [3,4] This case report is unique since Adult-onset SMA with concurrent PLA2R membranous nephropathy is rarely reported in the medical literature. We postulate that SMA gene defects cause genotoxic stress-inducing alteration in kidney structures, gene, and protein expression, [5] thereby contributing to podocytopathy and nephrotic syndrome. ...
2 0 2 3 I n d I a n J o u r n a l o f P a t h o l o g y a n d M I c r o b I o l o g y | P u b l I s h e d b y W o l t e r s K l u W e r-M e d K n o W ABSTRACT A 48-year-old male presented with proximal muscle weakness of the shoulder with difficulty in lifting objects above the shoulder in July 2012. Electromyogram was suggestive of chronic motor axonal loss with a myogenic pattern, and a deltoid muscle biopsy revealed groups of atrophic muscle fibers and hypertrophic fibers with pan fascicular atrophy suggestive of adult-onset spinal muscular atrophy. He was managed conservatively and developed bilateral pedal edema in August 2022. He had nephrotic range proteinuria, and renal biopsy revealed capillary wall thickening of glomeruli with serum PLA2R antibody positivity suggestive of primary membranous nephropathy. He was managed with telmisartan for 6 months, and there was a reduction in proteinuria. In January 2023, he was given Injection Rituximab due to worsening proteinuria. He failed to have a clinical resolution. This case report describes the management dilemma in membranous nephropathy with SMA due to the risk of toxic myopathies associated with tacrolimus and steroids.
... Treatment of Grave's-disease-associated MN has been successful with thiamazole, while others have reported improvement after radioiodine thyroid ablation but not after drug therapy [94][95][96]. However, in patients with myasthenia gravis and MN, treatment options include steroids, ACTH (which has been shown to achieve partial remission), RTX and, finally, thymectomy [97]. If celiac disease is the underlying cause, a trial of a gluten-free diet plus supportive therapy may lead to proteinuria remission, as described by Pestana et al. [98]. ...
Membranous nephropathy is an autoimmune disease affecting the glomeruli and is one of the most common causes of nephrotic syndrome. In the absence of any therapy, 35% of patients develop end-stage renal disease. The discovery of autoantibodies such as phospholipase A2 receptor 1, antithrombospondin and neural epidermal growth factor-like 1 protein has greatly helped us to understand the pathogenesis and enable the diagnosis of this disease and to guide its treatment. Depending on the complications of nephrotic syndrome, patients with this disease receive supportive treatment with diuretics, ACE inhibitors or angiotensin-receptor blockers, lipid-lowering agents and anticoagulants. After assessing the risk of progression of end-stage renal disease, patients receive immunosuppressive therapy with various drugs such as cyclophosphamide, steroids, calcineurin inhibitors or rituximab. Since immunosuppressive drugs can cause life-threatening side effects and up to 30% of patients do not respond to therapy, new therapeutic approaches with drugs such as adrenocorticotropic hormone, belimumab, anti-plasma cell antibodies or complement-guided drugs are currently being tested. However, special attention needs to be paid to the choice of therapy in secondary forms or in specific clinical contexts such as membranous disease in children, pregnant women and patients undergoing kidney transplantation.
