scholarly journals Successful immunosuppressive treatment and long-term follow-up of anti-Ri-associated paraneoplastic myelitis

2006 ◽  
Vol 77 (10) ◽  
pp. 1199-1200 ◽  
Author(s):  
F Leypoldt
2019 ◽  
Author(s):  
Anne-Marie Bogaert ◽  
Anne Hoorens ◽  
Marleen Praet ◽  
Jo Van Dorpe ◽  
Bruce Poppe ◽  
...  

Abstract We describe the case of a woman with minimal glomerular changes on initial kidney biopsy. On long-term follow-up, the patient developed nephrotic proteinuria and a second kidney biopsy was performed, which revealed focal segmental glomerulosclerosis (FSGS). Findings from electron microscopy (EM) examination suggested a genetic form of FSGS. Next-generation sequencing showed heterozygosity for a mutation in COL4A3. Collagen IV nephropathies can be linked to late-onset FSGS. By establishing a genetic cause of FSGS, immunosuppressive treatment can be avoided. This case emphasizes the importance of re-biopsy in cases of a non-explained rise in proteinuria. EM can be helpful in differentiating between primary and secondary FSGS and informing treatment strategies. In cases of adult-onset FSGS that cannot be categorized by clinical–pathological assessment, genetic testing should be considered.


Author(s):  
Marloes A. H. M. Michels ◽  
Kioa L. Wijnsma ◽  
Roel A. J. Kurvers ◽  
Dineke Westra ◽  
Michiel F. Schreuder ◽  
...  

Abstract Background C3 glomerulopathy (C3G) is a rare kidney disorder characterized by predominant glomerular depositions of complement C3. C3G can be subdivided into dense deposit disease (DDD) and C3 glomerulonephritis (C3GN). This study describes the long-term follow-up with extensive complement analysis of 29 Dutch children with C3G. Methods Twenty-nine C3G patients (19 DDD, 10 C3GN) diagnosed between 1992 and 2014 were included. Clinical and laboratory findings were collected at presentation and during follow-up. Specialized assays were used to detect rare variants in complement genes and measure complement-directed autoantibodies and biomarkers in blood. Results DDD patients presented with lower estimated glomerular filtration rate (eGFR). C3 nephritic factors (C3NeFs) were detected in 20 patients and remained detectable over time despite immunosuppressive treatment. At presentation, low serum C3 levels were detected in 84% of all patients. During follow-up, in about 50% of patients, all of them C3NeF-positive, C3 levels remained low. Linear mixed model analysis showed that C3GN patients had higher soluble C5b-9 (sC5b-9) and lower properdin levels compared to DDD patients. With a median follow-up of 52 months, an overall benign outcome was observed with only six patients with eGFR below 90 ml/min/1.73 m2 at last follow-up. Conclusions We extensively described clinical and laboratory findings including complement features of an exclusively pediatric C3G cohort. Outcome was relatively benign, persistent low C3 correlated with C3NeF presence, and C3GN was associated with higher sC5b-9 and lower properdin levels. Prospective studies are needed to further elucidate the pathogenic mechanisms underlying C3G and guide personalized medicine with complement therapeutics. Graphical abstract


2020 ◽  
pp. 112067212098187
Author(s):  
Andrea Guijarro ◽  
Nelida Muñoz ◽  
Nicolas Alejandre ◽  
Sheila Recuero ◽  
Olga Sanchez-Pernaute ◽  
...  

Acute zonal occult outer retinopathy (AZOOR) is a rare syndrome characterized by sudden onset of photopsia, scotomas, and abnormal electrophysiological tests, predominantly affecting young women. Although its pathogenesis remains unknown, auto-reactivity to retinal components is thought to mediate tissue damage. A 42-year-old woman presented with symptoms and examination consistent with the diagnosis of AZOOR. She was treated with azathioprine for 5 years. In spite of the immunosuppressive treatment, clear progression in the visual field, autofluorescence, electrophysiological tests and optical coherence tomography was observed. Treatment with intravenous immunoglobulins (IVIg) and subcutaneous Abatacept was subsequently started with little efficacy. Hereby, we present a case of progressive AZOOR despite aggressive immunosuppression with 10-year follow up. Currently, there is no consensus regarding management of AZOOR, and the convenience of administering aggressive immunosuppression remains uncertain.


2006 ◽  
Vol 42 (4) ◽  
pp. 277-282 ◽  
Author(s):  
Sebastien Behr ◽  
Laurent Cauzinille

Immune-mediated central nervous system inflammation is described in a series of 12 juvenile boxer dogs. A diagnosis of steroid-responsive meningitis-arteritis was made based on the clinical presentation and on diagnostic findings. The boxer breed was at a higher risk for this inflammatory condition than other breeds. Long-term follow-up (>2 years) confirmed a better prognosis in this breed than in the beagle and the Bernese mountain dog. Complete resolution of clinical signs without significant deficits or recurrences was obtained in all cases. Early clinical recognition and immunosuppressive treatment resulted in a better response and complete resolution of the disorder.


2019 ◽  
Vol 42 ◽  
Author(s):  
John P. A. Ioannidis

AbstractNeurobiology-based interventions for mental diseases and searches for useful biomarkers of treatment response have largely failed. Clinical trials should assess interventions related to environmental and social stressors, with long-term follow-up; social rather than biological endpoints; personalized outcomes; and suitable cluster, adaptive, and n-of-1 designs. Labor, education, financial, and other social/political decisions should be evaluated for their impacts on mental disease.


2001 ◽  
Vol 120 (5) ◽  
pp. A397-A397
Author(s):  
M SAMERAMMAR ◽  
J CROFFIE ◽  
M PFEFFERKORN ◽  
S GUPTA ◽  
M CORKINS ◽  
...  

2001 ◽  
Vol 120 (5) ◽  
pp. A204-A204
Author(s):  
B GONZALEZCONDE ◽  
J VAZQUEZIGLESIAS ◽  
L LOPEZROSES ◽  
P ALONSOAGUIRRE ◽  
A LANCHO ◽  
...  

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