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Sep 2024 DOI 10.14302/issn.2574-4488.jna-24-5219
V. Liang KellyCorresponding author
Primary membranous nephropathy (MN) is due to autoantibodies to phospholipase A2 receptor (PLA2R Ab). It is unclear whether COVID-19 vaccines can trigger flares of glomerular diseases such as primary MN. There have been increasing reports of glomerular diseases presenting or flaring after receipt of COVID-19 vaccines. We present a patient with primary MN who developed nephrotic syndrome after receiving her second mRNA-1273 COVID-19 vaccine with positive PLA2R Ab. Renal biopsy confirmed primary MN. She was treated for her primary MN flare with rituximab in a manner similar to non-vaccine-associated MN, which led to significant reduction in both PLA2R Ab level and proteinuria. This case adds to the growing literature on MN flares after receipt of mRNA COVID-19 vaccines. Close follow-up of patients with primary MN and other glomerular diseases after COVID-19 vaccination is warranted. Further research is needed to determine the pathophysiology behind vaccine-induced MN flares and whether there is a potential association between exposure to SARS-CoV-2 antigens and loss of tolerance to the PLA2R antigen.
Jan 2018 DOI 10.14302/issn.2470-5020.jnrt-17-1926
Nagel SimonCorresponding author
Department of Neurology, University of Heidelberg, Germany
Since the first description in 2013, 39 cases of anti-DPPX-encephalitis have been described. Main features of this autoimmune encephalitis characterized by antibodies against the potassium-channel-associated regulatory protein DPPX are gastrointestinal symptoms, cognitive dysfunction and signs of CNS hyperexcitability. While the majority of patients responds to immunotherapy relapses are frequent and often successfully treated with rituximab. Here we report another case of anti-DPPX-encephalitis presenting with the above mentioned triad. However, this is the first case of anti-DPPX-encephalitis in the context of a connective tissue disease combined with cerebral arteriopathy along with brain parenchymal lesions that we interpreted as a secondary, CTD-associated cerebral vasculitis. While the latter resolved under immunosuppressive treatment, comprising glucocorticosteroids, cyclophosphamide, rituximab and plasmapheresis, deterioration of the CTD and multiple infectious complications finally led to the patient's death. As histological evidence for cerebral vasculitis is lacking, other differential diagnoses for the observed cerebral arteriopathy, especially reversible cerebral vasoconstriction syndrome, have to be considered.
Dec 2016 DOI 10.14302/issn.2372-6601.jhor-16-1070
Sisi TianCorresponding author
Introduction: Post-transplant lymphoproliferative disease (PTLD) is a collection of conditions associated with abnormal proliferation of lymphoid tissues in patients after solid organ transplants (SOT). Its clinical presentations are quite variable and non-specific. Otolaryngological signs and symptoms, manifested as adenotonsillar hypertrophy or cervical lymphadenopathy, may guide to early detection and treatment. Methods: We conducted a retrospective review of all pediatric SOT recipients with the diagnosis of PTLD, age 0-18, between 2005 and 2014 at the Loma Linda University Children’s Hospital. The patient’s age, type of organ transplant, immunosuppression, head and neck signs and symptoms, imaging modality, EBV status, histology as well as treatment regimen information were recorded. Results: A total of 21 pediatric patients were included in this retrospective review with a history of solid organ transplant and a diagnosis of PTLD. The most commonly associated type of transplanted organ is heart (57.1%), followed by kidneys (33.3%) and liver (9.5%). Neck swelling (28.6%) was the main head and neck complaint while one patient developed upper airway obstruction with respiratory distress. Cervical lymphadenopathy was found in 66.7% and tonsillar hypertrophy in 9.5% of the patients. Monomorphic PTLD (46.2%) was the most common pathological diagnosis, followed by reactive hyperplasia (30.8%), Hodgkin lymphoma (15.4%) and polymorphic PTLD (7.7%). Majority of the PTLD patients were treated with rituximab and cyclophosphamide combination therapy with and without prednisone. Conclusion: Adenotonsillectomy and cervical lymph node biopsies are easy to perform with low complication rates. They serve an important role in the armamentarium in the early detection of PTLD in its early stage, allowing prompt treatment and prevention of further progression.
Jun 2014 DOI 10.14302/issn.2372-6601.jhor-14-378
Kulma-Kreft MonikaCorresponding author
Department of Radiotherapy and Medical Oncology, Gdynia Cancer Center, Gdynia
Progressive multifocal leukoencephalopathy (PML) is a rare complication associated, inter alia, with rituximab-based lymphoma treatment. PML diagnosis is made easier with the criteria recently published by the American Academy of Neurology. Unambiguous diagnosis of PML can be achieved by demonstration of the histopathological triad comprising:(1) demyelination, (2) bizarre astrocytes and (3) enlarged oligodendroglial nuclei together with detection of viral particles by electron microscopy. However, symptoms of PML may be similar to those observed during lymphoma progression into the central nervous system (CNS). Here we report the case of a patient with diffuse large B-cell lymphoma (DLBCL) treated with R-CHOP who developed clinical signs indicating PML. Intravital diagnostic methods failed to yield an unequivocal diagnosis of PML or lymphoma progression in the CNS. However, a post-mortem examination of brain biopsy specimens performed by electron microscopy demonstrated lesions typical for PML and the presence of viral particles. In addition, immunohistochemical assays identified a massive infiltration of lymphoma cells. The case thus suggests either the extremely rare coexistence of two complications: lymphoma CNS infiltration and PML or induction structural CNS lesions by lymphoma infiltration indistinguishable from PML. The presented findings thus highlight the need for a further review of the current diagnostic criteria for PML.