Diagnosis and successful management of calciphylaxis in a pancreas–kidney transplant patient

Sir,

We would like to refer to a case of fatal calciphylaxis developed 2 years after kidney transplantation published in the December 2004 issue of Nephrology Dialysis Transplantation [1].

We report a case of a 49-year-old woman who suffered from type 1 diabetes mellitus for 40 years. She became uraemic and underwent haemodialysis for 2 years, and finally received a pancreas–kidney transplant. Three months after transplantation, painful dry gangrene developed on her upper extremities and legs.

Two years after transplantation she was admitted with acral gangrene and osteomyelitis of the left hallux. Peripheral pulses were palpable, suggesting non-atherosclerotic lesions. The left hallux was amputated and histological examination showed all criteria of calciphylaxis. On admission, the patient had normal kidney and endocrine pancreas function (haemoglobin A1c, 4.9%; fructosamine, 222 µmol/l; BUN, 4.9 mmol/l; serum creatinine, 63 µmol/l). Oral glucose tolerance test and calculated insulin sensitivity indices [2] (ISIStumvoll = 0.14; HOMAIR = 2.26) showed increased insulin sensitivity. Extraosseal calcium-deposition in the connective tissues is characteristic for calciphylaxis [3]; however, in our case, bone scintigraphy and X-ray examinations were negative. Secondary hyperparathyroidism, a common cause of calciphylaxis was excluded (serum calcium, 1.99 mmol/l; phosphorous, 1.15 mmol/l; parathyroid hormone, 2.4 pmol/l). As hypofetuinaemia also predisposes to calciphylaxis [4], we measured serum fetuin level, which proved to be subnormal (408 µg/ml) [5]. Signs of autonomic neuropathy and day-time hypotension indicated sympathetic hypoactivity. The dose of methylprednisolone was increased to 24 mg/day [3]. The patient also received pentoxifylline, pentosan polysulfate and thioctic acid infusions on 5 consecutive days, which were repeated seven times in 2 years.

To our knowledge our patient was the first whose calciphylaxis was diagnosed 2 years after pancreas–kidney transplantation. Bone scintigraphy and X-ray were negative, parathormone level was normal. Fetuin deficiency with the consequent increased insulin sensitivity may explain both arterial medial calcification and intimal proliferation [4]. Sympathetic neuropathy, as well as increased insulin sensitivity, may cause vasodilation and thereby may lower the systolic blood pressure. Stenosis due to intimal proliferation—as a local factor—together with decreased perfusion pressure may lead to the development of ischaemic gangrene.

By improving the circulation with infusion therapy and increasing the dose of corticosteroid, all gangrene were healed, and 4 years after transplantation our patient is still alive.

Conflict of interest statement. None declared.

István Wittmann1, Péter Degrell1, Gergo A. Molnár1, Mónika Tamaskó1, Károly Kalmár Nagy2, Erzsébet Schmidt3, Eszter Fehér1, László Kalabay4, Boglárka Laczy1, László Wagner1, Zoltán Wagner1 and Judit Nagy1

1 Second Department of Medicine and Nephrological Center2 Surgical Department3 Department of Nuclear Medicine University of Pécs Hungary4 Third Department of Medicine Semmelweis University Budapest Hungary Email: istvan.wittmann{at}aok.pte.hu

References

  1. Vanbelleghem H, Terryn W, Van Leuven L, Van Caesbroeck D, Demetter P, Lameire N. A dramatic case of calciphylaxis 20 years after kidney transplantation. Nephrol Dial Transplant 2004; 19: 3183–3185[Free Full Text]
  2. Stumvoll M, Mitrakou A, Pimenta W et al. Use of the oral glucose tolerance test to assess insulin release and insulin sensitivity. Diabetes Care 2000; 23: 295–301[Abstract]
  3. Fine A, Zacharias J. Calciphylaxis is usually non-ulcerating: risk factors, outcome and therapy. Kidney Int 2002; 61: 2210–2217[CrossRef][ISI][Medline]
  4. Westenfeld R, Heiss A, Ketteler M, Floege J, Schwarz A, Jahnen-Dechent W. Calciphylaxis is linked to systemic deficiency of the calcification inhibitor AHSG/fetuin. Nephrol Dial Transplant 2002; 17 [Suppl 1]: 14
  5. Kalabay L, Jakab L, Prohászka Z et al. Human fetuin/{alpha}2HS-glycoprotein level as a novel indicator of liver cell function and short-term mortality in patients with liver cirrhosis and liver cancer. Eur J Gastroenterol Hepatol 2002; 14: 389–394[CrossRef][ISI][Medline]




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