calciphylaxis; uremic calcific arteriopathy/arteriolopathy
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Etiology
- hypercalcemia of any cause
- most common in patients with
- thrombophilia/hypercoagulable state frequently involved[12]
- warfarin may play role by reducing plasma protein C & plasma protein S
Epidemiology
- rare
- may occur more frequently in females
Pathology
- systemic medial calcification of medium-size arteries
- tissue calcification
- hyperparathyroidism, vitamin D supplementation, hyperphosphatemia play a role
- factors associated with uremia play a role may contribute
- histopathology
- arterial occlusion & calcification
- absence of vasculitis
- calcifications in subcutaneous tissue within necrotic lipocytes & within walls of small blood vessels[7]
- confluent epidermal necrosis[7]
* histopathology images[9]
Clinical manifestations
- precipitation by a specific event, such as skin trauma or injection
- painful subcutaneous nodules or plaques with red-brown discoloration (extremely painful)[7]
- areas of ischemic necrosis develop in dermis, subcutaneous fat, & less often muscle
- livedo reticularis develops
- painful &/or purpuric subcutaneous nodules &/or plaques with red-brown discoloration develop on areas with most adipose tissue: buttocks, trunk, breast, proximal extremities
- may form bullae
- progression of nodules & plaques to ischemic/necrotic ulcers
- black eschar formation in more advanced cases
- infection & superinfection of ulcers
- lesions may also appear on hands, fingers & lower extremities
* images[9]
Laboratory
- no diagnostic tests
- elevated serum PTH, serum phosporous, serum Ca+2 may be observed, but these are not always present
- elevated serum calcium x serum phosphate product
- skin biopsy
Radiology
- plain radiographs, computed tomography may be useful
- bone scintigraphy may be useful
* radiograph of hand[9]
Complications
- high mortality (up to 58%) due to infection
- 1 year survival is 37%[12]
- ischemic myopathy is uncommon
Differential diagnosis
- peripheral vascular disease
- vasculitis
- emboli
- cryoglobulinemia
- warfarin necrosis
- cellulitis
- nephrogenic systemic fibrosis
Management
- aggressive wound care
- pain management
- hydromorphone is metabolized by livec, cleared by hemodialysis[7]
- correction of Ca+2 & inorganic phosphate
- cessation of vitamin D supplementation
- treatment hyperphosphatemia with saline & sevelamer (RenaGel) rather than calcium acetate (PhosLo)
- sodium thiosulfate may be beneficial in some patients[7][12]
- cinacalcet has been successfully used[2]
- avoidance of local tissue trauma
- dialysis as needed
- consider withdrawal of immunosuppression in renal transplant patients refractory to interventions
- hyperbaric oxygen may be of some benefit[7][12]
- tissue plasminogen activator[12]
- parathyroidectomy[2][12]
- surgical debridement[12]
- prognosis is poor; response to therapy is uncertain
More general terms
References
- ↑ UpToDate 11.2 2003 http://www.uptodate.com
- ↑ 2.0 2.1 2.2 Essary LR and Wick MR Cutaneous calciphylaxis. An underrecognized clinicopathologic entity. Am J Clin Pathol 2000, 113:280 PMID: https://www.ncbi.nlm.nih.gov/pubmed/10664631
- ↑ Arch-Ferrer et al, Therapy for calciphylaxis: an outcome analysis. Surgery 2003, 134:941 PMID: https://www.ncbi.nlm.nih.gov/pubmed/14668726
- ↑ Janigan DT et al, Calcified subcutaneous arterioles with infarcts of the subcutis and skin ('calciphylaxic') in chronic renal failure. Am J Kidney Dis 200, 35:588 PMID: https://www.ncbi.nlm.nih.gov/pubmed/10739777
- ↑ Velasco N et al, Successful treatment of calciphylaxis with cinacalcet - An alternative treatment to parathyroidectomy? Nephrol Dial Transplant 2006, 21:1999 PMID: https://www.ncbi.nlm.nih.gov/pubmed/16603572
- ↑ Kale EK Metastatic calcification Dermatology Online Journal 9(4):2 , 2001 http://dermatology.cdlib.org/94/NYU/Nov2001/3.html
- ↑ 7.0 7.1 7.2 7.3 7.4 7.5 7.6 Medical Knowledge Self Assessment Program (MKSAP) 16, 17, 18, 19. American College of Physicians, Philadelphia 2012, 2015, 2018, 2021.
- ↑ Weenig RH, Sewell LD, Davis MD, McCarthy JT, Pittelkow MR. Calciphylaxis: natural history, risk factor analysis, and outcome. J Am Acad Dermatol. 2007 Apr;56(4):569-79. Epub 2006 Dec 1. PMID: https://www.ncbi.nlm.nih.gov/pubmed/17141359
- ↑ 9.0 9.1 9.2 9.3 Nunley JR, Elston DM (images) Medscape: Calciphylaxis http://emedicine.medscape.com/article/1095481-overview
- ↑ DermNet NZ: Calciphylaxis (images) http://www.dermnetnz.org/systemic/calciphylaxis.html
- ↑ Ross EA Evolution of treatment strategies for calciphylaxis. Am J Nephrol. 2011;34(5):460-7 PMID: https://www.ncbi.nlm.nih.gov/pubmed/21986387
- ↑ 12.0 12.1 12.2 12.3 12.4 12.5 12.6 12.7 McCarthy JT, El-Azhary RA, Patzelt MT et al Survival, Risk Factors, and Effect of Treatment in 101 Patients With Calciphylaxis. Mayo Clin Proc. 2016 Oct;91(10):1384-1394. PMID: https://www.ncbi.nlm.nih.gov/pubmed/27712637
El-Azhary RA, Patzelt MT, McBane RD et al Calciphylaxis: A Disease of Pannicular Thrombosis. Mayo Clin Proc. 2016 Oct;91(10):1395-1402. PMID: https://www.ncbi.nlm.nih.gov/pubmed/27712638 - ↑ Nigwekar SU, Thadhani R, Brandenburg VM. Calciphylaxis. N Engl J Med 2018; 378:1704-1714. May 3, 2018 PMID: https://www.ncbi.nlm.nih.gov/pubmed/29719190 https://www.nejm.org/doi/full/10.1056/NEJMra1505292
- ↑ Nigwekar SU, Kroshinsky D, Nazarian RM et al Calciphylaxis: risk factors, diagnosis, and treatment. Am J Kidney Dis. 2015 Jul;66(1):133-46. Review. PMID: https://www.ncbi.nlm.nih.gov/pubmed/25960299 Free PMC Article