Calciphylaxis and Kidney Disease: A Review

Marin, Benjamin Gallo; Aghagoli, Ghazal; Hu, Susie L.; Massoud, Cathy M.; Robinson‐Bostom, Leslie

doi:10.1053/j.ajkd.2022.06.011

Cited by 29 publications

(28 citation statements)

References 83 publications

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“…Apart from deposits of calcium apatite in the tunica media of cutaneous vessels, associated with fibrosis of the tunica intima and subsequent stenosis, coagulation disorders and thrombotic stenosis are also thought to play a role in the pathophysiology. 55,56 A prothrombotic coagulation status appears to be associated with calciphylaxis. 57 Chronic inflammation is also considered a potential trigger of extraossary calcification.…”

Section: Pathophysiologymentioning

confidence: 99%

“…Risk factors for the occurrence of calciphylaxis (after Gallo Marin et al Calciphylaxis and Kidney Disease: A Review) 56. Disorders of the calcium-phosphate metabolism (for example in the context of kidney disease, bone disease, tumors, or due to medication) (Secondary) hyperparathyreoidism Increase of alkaline phosphatase, calcium-phosphate product > 70 mg 2 /dl2 …”

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confidence: 99%

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Occlusive cutaneous vasculopathies as cause of chronic ulcers

Ronicke,

Berking,

Erfurt‐Berge

2024

J Deutsche Derma Gesell

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SummaryThe term occluding vasculopathies covers a large number of different conditions. These often manifest as skin ulcers. Occluding vasculopathies should be considered in the differential diagnosis of leg ulcers. The term “occlusive vasculopathies” encompasses pathophysiologically related entities that share structural or thrombotic obliteration of small cutaneous vessels. In this article, we will focus on livedoid vasculopathy with and without antiphospholipid syndrome and calciphylaxis with differentiation from hypertonic leg ulcer as the most relevant differential diagnoses of leg ulcer. The term also includes vascular occlusion, for example due to oxalate or cholesterol embolism, and septic vasculopathy. This often leads to acral ulceration and is therefore not a differential diagnosis with classic leg ulcers. It will not be discussed in this article.Occlusive vasculopathy may be suspected in the presence of the typical livedo racemosa or (non‐inflammatory) retiform purpura as a sign of reduced cutaneous perfusion in the wound area. Inflammatory dermatoses, especially vasculitides, must be differentiated. This is achieved by histopathological evaluation of a tissue sample of sufficient size and depth taken at the appropriate time. In addition, specific laboratory parameters, particularly coagulation parameters, can support the diagnosis.

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Section: Pathophysiologymentioning

confidence: 99%

mentioning

confidence: 99%

Occlusive cutaneous vasculopathies as cause of chronic ulcers

Ronicke,

Berking,

Erfurt‐Berge

2024

J Deutsche Derma Gesell

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“…Weitere Imitatoren von Vaskulitiden mittelgroßer (und kleiner) Gefäße sind das Cholesterin-Embolie-Syndrom (das vor Allem nach Angiographien oder gefäßchirurgischen Eingriffen eintreten kann) [32] und die in den meisten Fällen mit chronischer oder akuter Niereninsuffizienz assoziierte Kalziphylaxie [33], einer lebensbedrohlichen Erkrankung, bei der es zu Ablagerungen von Kalzium und Phosphat in den Gefäßen von Haut und Fettgewebe mit nachfolgender Vaskulitis, Pannikulitis und Nekrose kommt.…”

Section: Weitere Imitatoren Von Vaskulitiden Mit Befall Insbesondere ...unclassified

Vaskulitis mimics

Schirmer

Both

Müller

2023

Aktuelle Rheumatologie

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ZusammenfassungIdiopathische Vaskulitiden sind seltene entzündliche Systemerkrankungen, die nach der Chapel-Hill Konsensus-Nomenklatur nach der Größe der prädominant betroffenen Gebiete von Blutgefäßen (große, mittelgroße, kleine Gefäße und Gefäße variabler Größe) eingeteilt werden. Vaskulitis mimics sind Syndrome, die ein ähnliches klinisches Bild hervorrufen oder leicht mit einer idiopathischen Vaskulitis verwechselt werden und teils sogar ein Krankheitsbild, das klinisch und histologisch einer Vaskulitis gleicht, auslösen können. Die Zahl der Vaskulitis mimics ist groß, je nach betroffenem Gefäßgebiet kommen hereditäre Erkrankungen des Bindegewebes, genetisch bedingte Immundefekt- und Autoinflammationssyndrome, infektiöse Erkrankungen, seltene entzündliche Systemerkrankungen, Tumorerkrankungen, medikamenteninduzierte Syndrome und zahlreiche weitere infrage. In diesem Review wird eine Auswahl klassischer Imitatoren von Vaskulitiden, orientiert an der Größe der betroffenen Blutgefäße präsentiert und Konstellationen, die typische „Fallstricke“ in der klinischen Abklärung darstellen können, diskutiert.

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Section: Introductionmentioning

confidence: 99%

“…Calciphylaxis is a rare but serious disorder of vascular calcification typically presenting with painful skin ulcerations that predominantly affects patients with advanced chronic kidney disease (CKD). 1 There is no approved therapy for calciphylaxis. A 2022 meta-analysis of 6 clinical trials 2 concluded that intravenous sodium thiosulfate (STS) could attenuate the progression of macrovascular calcification (in the coronary and iliac arteries) among hemodialysis patients.…”

Section: Introductionmentioning

confidence: 99%

Intravenous Sodium Thiosulphate for Calciphylaxis of Chronic Kidney Disease

et al. 2023

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ImportanceCalciphylaxis is a rare disease with high mortality mainly involving patients with chronic kidney disease (CKD). Sodium thiosulphate (STS) has been used as an off-label therapeutic in calciphylaxis, but there is a lack of clinical trials and studies that demonstrate its effect compared with those without STS treatment.ObjectiveTo perform a meta-analysis of the cohort studies that provided data comparing outcomes among patients with calciphylaxis treated with and without intravenous STS.Data SourcesPubMed, Embase, Cochrane Library, Web of Science, and ClinicalTrials.gov were searched using relevant terms and synonyms including sodium thiosulphate and calci* without language restriction.Study SelectionThe initial search was for cohort studies published before August 31, 2021, that included adult patients diagnosed with CKD experiencing calciphylaxis and could provide a comparison between patients treated with and without intravenous STS. Studies were excluded if they reported outcomes only from nonintravenous administration of STS or if the outcomes for CKD patients were not provided.Data Extraction and SynthesisRandom-effects models were performed. The Egger test was used to measure publication bias. Heterogeneity was assessed using the I2 test.Main Outcomes and MeasuresSkin lesion improvement and survival, synthesized as ratio data by a random-effects empirical Bayes model.ResultsAmong the 5601 publications retrieved from the targeted databases, 19 retrospective cohort studies including 422 patients (mean age, 57 years; 37.3% male) met the eligibility criteria. No difference was observed in skin lesion improvement (12 studies with 110 patients; risk ratio, 1.23; 95% CI, 0.85-1.78) between the STS and the comparator groups. No difference was noted for the risk of death (15 studies with 158 patients; risk ratio, 0.88; 95% CI, 0.70-1.10) and overall survival using time-to-event data (3 studies with 269 participants; hazard ratio, 0.82; 95% CI, 0.57-1.18). In meta-regression, lesion improvement associated with STS negatively correlated with publication year, implying that recent studies are more likely to report a null association compared with past studies (coefficient = −0.14; P = .008).Conclusions and RelevanceIntravenous STS was not associated with skin lesion improvement or survival benefit in patients with CKD experiencing calciphylaxis. Future investigations are warranted to examine the efficacy and safety of therapies for patients with calciphylaxis.

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Calciphylaxis and Kidney Disease: A Review

Cited by 29 publications

References 83 publications

Occlusive cutaneous vasculopathies as cause of chronic ulcers

Occlusive cutaneous vasculopathies as cause of chronic ulcers

Vaskulitis mimics

Intravenous Sodium Thiosulphate for Calciphylaxis of Chronic Kidney Disease

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