|Year : 2015 | Volume
| Issue : 2 | Page : 139-141
Extensive vascular calcification
Mukesh Dhillon1, Vipin Kwatra2, Saurabh Dawra3, K. V. S. Hari Kumar3
1 Department of Medicine, Military Hospital, Ambala, Punjab, India
2 Department of Surgery, Military Hospital, Ambala, Punjab, India
3 Department of Endocrinology, Command Hospital, Chandimandir, Panchkula, Haryana, India
|Date of Web Publication||17-Jan-2016|
K. V. S. Hari Kumar
Department of Endocrinology, Command Hospital, Chandimandir, Panchkula, Haryana
Source of Support: None, Conflict of Interest: None
Calciphylaxis denotes the presence of cutaneous gangrene secondary to the ischemic necrosis caused by vascular calcification. This is seen in patients with end stage renal disease, especially those using the renal replacement therapy. We recently encountered an elderly patient with long standing type 2 diabetes who presented with diabetic foot disease. The evaluation revealed extensive vascular calcifications along with diabetic nephropathy. In this report, we present the case details along with the interesting aspects of the calciphylaxis.
Keywords: Calciphylaxis, diabetic nephropathy, type 2 diabetes, vascular calcification
|How to cite this article:|
Dhillon M, Kwatra V, Dawra S, Kumar KH. Extensive vascular calcification. Indian J Health Sci Biomed Res 2015;8:139-41
| Introduction|| |
A syndrome of arteriolar calcification coupled with the tissue necrosis is termed as the calciphylaxis. This is also known as the calcific uremic arteriolopathy, due to the commonest association with patients of chronic kidney disease (CKD).  In addition to the CKD, calciphylaxis is described in patients with lymphoma, leukemia, myeloma and other causes of hyperparathyroidism.  The disease usually involves the small and medium arterioles with sparing of the large vessels. The presence of calciphylaxis is an indicator of poor prognosis. The mortality rate is in excess of 80% in these patients and they usually succumb to the underlying infection and secondary sepsis. 
Calciphylaxis is reported to be due to the exaggerated sensitivity of the vascular tissue to the alterations in the mineral metabolism. The pathogenesis of the calciphylaxis is not understood clearly due to its varied presentation. The risk factors for calciphylaxis are old age, smoking, hyperparathyroidism, diabetes, dyslipidemia and elevated calcium and phosphorus product (CaPP).  The disease presents with gangrene of the terminal portions of the body like penis, toes and fingers.  Calcification of the vasculature results in the luminal narrowing and reduction of the blood supply leading to ischemic necrosis of the tissues. We present a case of calciphylaxis along with extensive vascular calcifications and asymptomatic kidney disease with an uneventful recovery.
| Case Report|| |
A 60-year-old patient, known case of type 2 diabetes mellitus (T2DM) for 12 years presented with a nonhealing ulcer of right foot for the duration of 1-month. The patient denied history of claudication pain and any similar ulcer in the past. His glycemic monitoring was poor and he denied any features to suggest macrovascular or microvascular complications of the diabetes. General examination revealed normotensive individual with no significant systemic findings. Local examination revealed cellulitis of right foot with an ulcer in between the great toe and second toe. The ulcer had a necrotic base, punched out, tender margins with dry and discolored skin around it. All the peripheral pulses were palpable and the rest of the systemic examination was normal.
His investigations revealed anemia (Hb - 10 gm/dL), polymorphonuclear leucocytosis (total leucocyte count - 24,900/cumm), azotemia (urea - 68 mg/dL, creatinine - 2 mg/dL), dyslipidemia (total cholesterol 225 mg/dL, LDL - cholesterol - 156 mg/dL, triglycerides - 223 mg/dL) and poor glycemic control (glycosylated haemoglobin - 9.2%). A detailed work up for the diabetic complications revealed background retinopathy and nephropathy (macroalbuminuria 480 mg/24 h). Foot radiograph did not show any evidence of osteomyelitis but did show the presence of calcified dorsalis pedis artery [Figure 1], (panel A). Screening radiographs showed extensive calcification of popliteal (panel B), femoral and iliac (panel C), radial and ulnar arteries (panel D). Work up for calciphylaxis revealed normal calcium (9.2 mg/dL), elevated phosphorus (5 mg/dL), normal parathormone (16.2 pg/ml) and normal CaPP (46).
|Figure 1: Radiographs showing calcification of dorsalis pedis, (a) Popliteal, (b) Femoral, iliac, (c) Radial and ulnar (d) Arteries|
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He was diagnosed as a case of calciphylaxis and treated with wound debridement, daily dressing, broad spectrum antibiotics, sevelamer, calcium, Vitamin D and other supportive measures. Glycemic control was optimized with the use of multiple doses of insulin along with linagliptin. The wound healed completely over next 8 weeks with no residual complication. During the last review, his HbA1c improved to 7.8% and the patient remained symptom free.
| Discussion|| |
Our patient had many atypical features in his presentation. He had stage three CKD (estimated glomerular filtration rate of 46 ml/min/1.73 m 2 ) at the time of presentation of such an extensive network of vascular calcifications. Another feature is the relative lack of tissue necrosis despite the presence of significant vascular calcifications except for the diabetic foot disease.
Calciphylaxis is a complex syndrome of arterial calcification and tissue necrosis in patients of end stage renal disease or undergoing renal replacement therapy.  Extensive arterial calcification is reported in patients with diabetes, dyslipidemia and disorders of mineral metabolism. Other risk factors for calciphylaxis are female sex, obesity, high pulse pressure, protein C deficiency and use of glucocorticoids. The clinical presentation includes mottled skin, black eschar formation and nonhealing ulceration.  Our patient had all the typical features of the calciphylaxis in his presentation.
Calciphylaxis is a marker of high mortality and the majority of patients succumb due to secondary infection and severe sepsis.  The management strategies include reduction of the calcium phosphorus product, aggressive wound care, local and systemic antibiotics and anti-inflammatory therapy.  Sodium thiosulfate is recently introduced as an excellent option for patients with calciphylaxis. The drug binds to the calcium, reduces the CaPP along with reduction of the oxidative stress. 
| Conclusion|| |
To conclude, we report an interesting case of extensive vascular calcification in a patient with T2DM and early stage of CKD. Our report highlights the importance of screening for disorders of mineral metabolism in all patients of diabetic foot disease and nonhealing ulcers.
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Conflicts of interest
There are no conflicts of interest.
| References|| |
Lee JL, Naguwa SM, Cheema G, Gershwin ME. Recognizing calcific uremic arteriolopathy in autoimmune disease: An emerging mimicker of vasculitis. Autoimmun Rev 2008;7:638-43.
Nigwekar SU, Wolf M, Sterns RH, Hix JK. Calciphylaxis from nonuremic causes: A systematic review. Clin J Am Soc Nephrol 2008;3:1139-43.
Agarwal MM, Singh SK, Mandal AK. Penile gangrene in diabetes mellitus with renal failure: A poor prognostic sign of systemic vascular calciphylaxis. Indian J Urol 2007;23:208-10.
Nigwekar SU, Kroshinsky D, Nazarian RM, Goverman J, Malhotra R, Jackson VA, et al.
Calciphylaxis: Risk factors, diagnosis, and treatment. Am J Kidney Dis 2015;66:133-46.
Karpman E, Das S, Kurzrock EA. Penile calciphylaxis: Analysis of risk factors and mortality. J Urol 2003;169:2206-9.
Zhou Q, Neubauer J, Kern JS, Grotz W, Walz G, Huber TB. Calciphylaxis. Lancet 2014;383:1067.
Parker RW, Mouton CP, Young DW, Espino DV. Early recognition and treatment of calciphylaxis. South Med J 2003;96:53-5.
Yu Z, Gu L, Pang H, Fang Y, Yan H, Fang W. Sodium thiosulfate: An emerging treatment for calciphylaxis in dialysis patients. Case Rep Nephrol Dial 2015;5:77-82.