Castleman disease and SLE in a G6PD-deficient Marfan patient: a case report and literature review

We report a 15-year-old Caucasian G6PD deficient Marfan male patient, who presented with tonic –clonic seizures, fever, a hemolytic episode, and general symptoms. After the discovery of hepatosplenomegaly, malar rash, and painless lymphadenopathy, further testing diagnosed a multifocal Castleman disease of the hyaline vascular subtype and systemic lupus erythematosus with lupus nephritis th at got 35 points on the 2019 EULAR/ACR criteria. G6PD deficiency, SLE&Castleman disease, and seizures were handled medically with eventual improvement in the patient ’s condition.Discussion and conclusionIt is extremely rare to discover the gathering of these four diseases in the same patient. Marfan syndrome and G6PD deficiency were proven by respective clinical and laboratory examinations. Castleman disease that tends to occur in older age groups was confirmed via pathological study of a lymph node biopsy, which was compatible with the HHV-8 negative type reported in Asian countries. SLE is part of the differential diagnosis for Castleman disease, yet the newest evidence strongly supports its presence as a distinct entity. However, no concrete proof is available to suggest a causative relationship between the four of them, rather than a coincidental occurrence.
Source: Autoimmunity Highlights - Category: Allergy & Immunology Source Type: research

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ConclusionThese results  suggest that miR-1968-5p may be involved in the pathogenesis of lupus nephritis of NZBWF1 mice by targeting csf1.
Source: Clinical and Experimental Nephrology - Category: Urology & Nephrology Source Type: research
CONCLUSION: The present study explored the potential targets and signaling pathways of curcumin against SLE-ONFH, which could provide a better understanding of its effects in terms of regulating cell cycle, angiogenesis, immunosuppression, inflammation, and bone destruction.PMID:34557547 | PMC:PMC8455200 | DOI:10.1155/2021/5538643
Source: Biomed Res - Category: Research Authors: Source Type: research
CONCLUSION: We found a more severe form of clinical manifestation in pediatric SLE patients at the time of the first presentation in the form of severe renal and extrarenal manifestation compared to other parts of the country.PMID:34558344 | DOI:10.1177/09612033211045069
Source: Lupus - Category: Rheumatology Authors: Source Type: research
CONCLUSIONS: Multiple PIDs can lead to monogenic lupus. Different PID-related monogenic lupus has different suitable targeted drugs.PMID:34559261 | DOI:10.1007/s00011-021-01479-6
Source: Cell Research - Category: Cytology Authors: Source Type: research
J Nephrol. 2021 Sep 24. doi: 10.1007/s40620-021-01144-5. Online ahead of print.NO ABSTRACTPMID:34559399 | DOI:10.1007/s40620-021-01144-5
Source: Journal of Nephrology - Category: Urology & Nephrology Authors: Source Type: research
ConclusionsMultiple PIDs can lead to monogenic lupus. Different PID-related monogenic lupus has different suitable targeted drugs.
Source: Inflammation Research - Category: Research Source Type: research
Date: September 23, 2021 Issue #:  1634Summary:  The B-lymphocyte stimulator (BLyS)-specific inhibitor belimumab (Benlysta– GSK), which was approved earlier for treatment of active, autoantibody-positive, nonrenal, systemic lupus erythematosus (SLE), has now been approved for use in addition to standard therapy for treatment of active lupus nephritis in adults. Belimumab is the first drug to be approved in the US for treatment of both SLE and lupus nephritis.
Source: The Medical Letter - Category: Drugs & Pharmacology Authors: Tags: Azathioprine belimumab Benlysta Cellcept corticosteroids Cyclophosphamide Cytoxan Imuran Lupkynis Lupus Mycophenolate mofetil Prograf Rituxan Rituximab Tacrolimus voclosporin Source Type: research
Lupus. 2021 Sep 23:9612033211048048. doi: 10.1177/09612033211048048. Online ahead of print.NO ABSTRACTPMID:34551646 | DOI:10.1177/09612033211048048
Source: Lupus - Category: Rheumatology Source Type: research
CONCLUSION: Results of this meta-analysis showed that antimalarial drugs might be protective factors for cancer in SLE. Hydroxychloroquine might be a protective factor for cancer in SLE patients.KEY MESSAGESAntimalarials might be protective factors for cancer in SLE.Hydroxychloroquine might be a protective factor for cancer in SLE patients.The first article to perform the meta-analysis of antimalarial drugs on the risk of cancer in SLE patients.PMID:34553648 | DOI:10.1080/07853890.2021.1981547
Source: Annals of Medicine - Category: Internal Medicine Authors: Source Type: research
In this study we investigated whether NCTD exerted therapeutic effects in a mouse SLE model. Lupus prone female MRL/lpr mice were treated with NCTD (1, 2 mg·kg-1·d-1, ip) for 8 weeks. We showed that NCTD administration significantly decreased mortality rate, diminished the expression of anti-dsDNA IgG antibody, a diagnostic marker for SLE, as well as restored renal structure and function in MRL/lpr mice. Moreover, NCTD administration dose-dependently inhibited lymphoproliferation and T cell accumulation in the spleens of MRL/lpr mice. We further revealed that NCTD specifically inhibited DN T cell proliferatio...
Source: Acta Pharmacologica Sinica - Category: Drugs & Pharmacology Authors: Source Type: research
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