TY - JOUR
T1 - Calcific uremic arteriolopathy (calciphylaxis)
T2 - Case series and review of clinical features and terminology
AU - Rickels, Michael R.
AU - Junkins-Hopkins, Jacqueline M.
AU - Metkus, Thomas S.
AU - Iqbal, Nayyar
PY - 2007/1/1
Y1 - 2007/1/1
N2 - We present 3 cases of calciphylaxis, better termed calcific uremic arteriolopathy (CUA), which illustrate typical and atypical presentations of this disorder to discuss its classification, pathophysiology, and treatment based on a review of the literature. Of the 3 patients presenting with biopsy-proven CUA, 1 patient with proximal lesions expired in the setting of upper gastrointestinal bleeding and superinfection of ulcers, a second patient had an extensive distal ulcerative lesion that failed to heal and necessitated lower-extremity amputation, and a third patient with multiple small distal lesions experienced complete resolution of the ulcers. The classification, pathophysiology, and treatment of CUA are detailed here. CUA is a heterogeneous syndrome which typically follows a progressive course leading to loss of life or limb. Diagnosis requires a high index of suspicion and low threshold for biopsy in at-risk patients. Parathyroidectomy is appropriate to rapidly lower the calcium-phosphorus product in the setting of marked hyperparathyroidism; additional therapy includes the avoidance of purported precipitants, medical lowering of the serum calcium and phosphorus, and promotion of wound healing with hyperbaric oxygen therapy.
AB - We present 3 cases of calciphylaxis, better termed calcific uremic arteriolopathy (CUA), which illustrate typical and atypical presentations of this disorder to discuss its classification, pathophysiology, and treatment based on a review of the literature. Of the 3 patients presenting with biopsy-proven CUA, 1 patient with proximal lesions expired in the setting of upper gastrointestinal bleeding and superinfection of ulcers, a second patient had an extensive distal ulcerative lesion that failed to heal and necessitated lower-extremity amputation, and a third patient with multiple small distal lesions experienced complete resolution of the ulcers. The classification, pathophysiology, and treatment of CUA are detailed here. CUA is a heterogeneous syndrome which typically follows a progressive course leading to loss of life or limb. Diagnosis requires a high index of suspicion and low threshold for biopsy in at-risk patients. Parathyroidectomy is appropriate to rapidly lower the calcium-phosphorus product in the setting of marked hyperparathyroidism; additional therapy includes the avoidance of purported precipitants, medical lowering of the serum calcium and phosphorus, and promotion of wound healing with hyperbaric oxygen therapy.
KW - Calcium phosphorus product
KW - Chronic kidney disease
KW - Cutaneous ulceration
KW - Hyperparathyroidism
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U2 - 10.1097/01.ten.0000255808.25351.45
DO - 10.1097/01.ten.0000255808.25351.45
M3 - Review article
AN - SCOPUS:33846829760
SN - 1051-2144
VL - 17
SP - 57
EP - 62
JO - Endocrinologist
JF - Endocrinologist
IS - 1
ER -