Calciphylaxis and nephrogenic fibrosing dermopathy with pseudoxanthoma elasticum‐like changes: Successful treatment with sodium thiosulfate

Ishikawa, M.; Motegi, Sei‐ichiro; Toki, Sayaka; Endo, Yukie; Yasuda, Masahito; Ishikawa, Osamu

doi:10.1111/1346-8138.14780

Cited by 5 publications

(4 citation statements)

References 5 publications

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“…Eventually, 69 studies were assessed for full-text eligibility, out of which 39 were eventually included in our review. 10 , 11 , 18–54 From these 39 studies, 21 were case reports, 16 were case series, and 2 were retrospective studies. Figure 1 summarizes each stage.…”

Section: Resultsmentioning

confidence: 99%

The Clinical Manifestations and Efficacy of Different Treatments Used for Nephrogenic Systemic Fibrosis: A Systematic Review

Farooqi

Mumtaz

Arif

et al. 2023

IJNRD

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Aim Nephrogenic systemic fibrosis (NSF) is a rare disorder that occurs in association majorly with chronic kidney disease (CKD). The lack of collective quantitative data on its clinical manifestations and the different treatment options’ efficacy, call the need for our investigation. Methods A systematic review was conducted covering a timeline from inception up to July 2022 without any restrictions. Article screening and data extraction were performed independently on PubMed, Google Scholar, ScienceDirect, and Cochrane Library. The keywords that we used were CKD, NSF, Gadolinium enduced fibrosis, etc; shortlisted articles were assessed for risk of bias. Data were presented as frequencies and percentages, with a confidence interval of 95%. A chi-square test was also done to find significant relationships, with a p -value <0.05 considered significant. Results We had 83 patients in this review consisting of 44 (55.7%) females with a mean age of 51.4±14.6 years. Sixty-nine (83.1%) patients had chronic kidney disease predisposition to NSF. Previous exposure to gadolinium-based contrast dyes was seen in 66 (79.5%) patients). The most common symptom in patients was cutaneous lesions in 69 (83.1%) patients. The most used treatments were ultraviolet therapy, renal transplant, and extracorporeal photopheresis; in 13.3% of the patients each. Condition in most patients either improved (67.1%) or remained stable (11.8%). Chi-square testing found that the treatments offered were also seen to be significantly related to outcome (p=0.015). Conclusion The findings in this study provide a quantitative measurement of NSF’s presentations and treatment efficacies. This serves to make way for researchers to form comprehensive guidelines on the presentation-based treatment of NSF.

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Section: Resultsmentioning

confidence: 99%

The Clinical Manifestations and Efficacy of Different Treatments Used for Nephrogenic Systemic Fibrosis: A Systematic Review

Farooqi

Mumtaz

Arif

et al. 2023

IJNRD

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“…On the other hand, relatively few case reports have described the effect of STS for calciphylaxis in Japanese patients [8][9][10][11][12][13] . STS was effective in all of the previously reported Japanese patients who were undergoing dialysis.…”

Section: Discussionmentioning

confidence: 99%

“…In two case reports, STS (10 g/ day) was administered 3 times/week during dialysis 10,11) . Conversely, Tokashiki et al and Ishikawa et al administered 20 and 5-25 g/day, respectively 9,13) . The treatment was discontinued in the latter due to side effects, such as metabolic acidosis, anorexia, and nausea 12) .…”

Section: Discussionmentioning

confidence: 99%

Efficacy and Safety of Sodium Thiosulfate in Japanese Patients with Calciphylaxis

Nishiyama

Ueno

Nishiguchi

et al. 2021

International Journal of Surgical Wound Care

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Calciphylaxis is a rare disease histopathologically characterized by calcification of the medial layer of small cutaneous vessels, resulting in painful and intractable skin ulcerations in the legs. The disease is potentially lifethreatening, with more than 50% of patients dying within 1 year of diagnosis 1). Secondary infections and organ failure are also possible complications. Patients with end-stage renal disease are most susceptible to calciphylaxis; however, calciphylaxis may also affect patients with hypoparathyroidism requiring calcium salts or vitamin D, those with obesity, and those using warfarin. Ever since Cicone et al. 2) reported the successful treatment of calciphylaxis with intravenous sodium thiosulfate (STS), STS therapy has been commonly used as the first-line treatment for this disease, showing beneficial effects in most patients 3). However, systemic STS may have various side effects, such as nausea, vomiting, hypocalcemia, metabolic acidosis, and QTinterval prolongation. According to several recent reports, intralesional STS may prevent systemic side effects, but it may cause pain during injection. Although the use of STS has been described in case reports, case series, and in a single retrospective review, the effects and side effects of STS on calciphylaxis have not been confirmed by blinded randomized clinical studies. Therefore, a standardized treatment protocol for STS has not been

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“…Its administration in end-stage renal disease patients resulted in a significantly reduced calcification of the iliac artery and cardiac valves [ 218 ]. As NaTS has also been effectively applied in the treatment of patients with calciphylaxis [ 219 , 220 , 221 ], it has recently been administered to a patient with severe and early onset PXE due to PXE/GACI-overlap-syndrome [ 144 ]. After six months, calcific stenoses of the coeliac and mesenteric arteries were no longer detectable; clinical symptoms such as pre- and postprandial pain were resolved.…”

Section: Therapeutic Approachesmentioning

confidence: 99%

Therapy of Pseudoxanthoma Elasticum: Current Knowledge and Future Perspectives

et al. 2021

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Pseudoxanthoma elasticum (PXE) is a rare, genetic, metabolic disease with an estimated prevalence of between 1 per 25,000 and 56,000. Its main hallmarks are characteristic skin lesions, development of choroidal neovascularization, and early-onset arterial calcification accompanied by a severe reduction in quality-of-life. Underlying the pathology are recessively transmitted pathogenic variants of the ABCC6 gene, which results in a deficiency of ABCC6 protein. This results in reduced levels of peripheral pyrophosphate, a strong inhibitor of peripheral calcification, but also dysregulation of blood lipids. Although various treatment options have emerged during the last 20 years, many are either already outdated or not yet ready to be applied generally. Clinical physicians often are left stranded while patients suffer from the consequences of outdated therapies, or feel unrecognized by their attending doctors who may feel uncertain about using new therapeutic approaches or not even know about them. In this review, we summarize the broad spectrum of treatment options for PXE, focusing on currently available clinical options, the latest research and development, and future perspectives.

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Calciphylaxis and nephrogenic fibrosing dermopathy with pseudoxanthoma elasticum‐like changes: Successful treatment with sodium thiosulfate

Cited by 5 publications

References 5 publications

The Clinical Manifestations and Efficacy of Different Treatments Used for Nephrogenic Systemic Fibrosis: A Systematic Review

The Clinical Manifestations and Efficacy of Different Treatments Used for Nephrogenic Systemic Fibrosis: A Systematic Review

Efficacy and Safety of Sodium Thiosulfate in Japanese Patients with Calciphylaxis

Therapy of Pseudoxanthoma Elasticum: Current Knowledge and Future Perspectives

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