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Case Reports
. 2021 Apr-Jun;43(2):274-278.
doi: 10.1590/2175-8239-JBN-2020-0025.

Multiple extremity necrosis in fatal calciphylaxis: Case report

[Article in English, Portuguese]
Diego Ennes Gonzalez  1 , Renato Demarchi Foresto  1 , Ana Luiza Santos Maldonado  1 , Wallace Stwart Carvalho Padilha  1 , Fernanda Badiani Roberto  1 , Maria Eduarda Vilanova da Costa Pereira  1 , Marcelino de Souza Durão Junior  2 , Aluizio Barbosa Carvalho  2
Affiliations
Case Reports

Multiple extremity necrosis in fatal calciphylaxis: Case report

[Article in English, Portuguese]
Diego Ennes Gonzalez et al. J Bras Nefrol. 2021 Apr-Jun.

Abstract
in English, Portuguese

Introduction: The clinical impact of vascular calcification is well established in the context of cardiovascular morbidity and mortality, but other clinical syndromes, such as calciphylaxis, although less frequent, have a significant impact on chronic kidney disease.

Methods: Case report of a 27-year-old woman, who had complained of bilateral pain in her toes for 3 days, with the presence of small necrotic areas in the referred sites. She had a history of type 1 diabetes (25 years ago), with chronic kidney disease, on peritoneal dialysis, in addition to rheumatoid arthritis. She was admitted to the hospital, which preceded the current condition, due to exacerbation of rheumatoid arthritis, evolving with intracardiac thrombus due to venous catheter complications, when she started using warfarin. Ischemia progressed to her feet, causing the need for bilateral amputations. Her chirodactyls were also affected. Thrombophilia, vasculitis, endocarditis or other embolic sources were investigated and discarded. Her pathology report evidenced skin necrosis and superficial soft parts with recent arterial thrombosis, and Monckeberg's medial calcification. We started treatment with bisphosphonate and sodium thiosulfate, conversion to hemodialysis and replacement of warfarin with unfractionated heparin. Despite all the therapy, the patient died after four months of evolution.

Discussion: Calciphylaxis is a rare microvasculature calcification syndrome that results in severe ischemic injuries. It has pathogenesis related to the mineral and bone disorder of chronic kidney disease combined with the imbalance between promoters and inhibitors of vascular calcification, with particular importance to vitamin K antagonism.

Conclusion: The preventive strategy is fundamental, since the therapy is complex with poorly validated effectiveness.

Introdução:: O impacto clínico da calcificação vascular está bem estabelecido no âmbito de morbimortalidade cardiovascular, mas outras síndromes clínicas, como a calcifilaxia, apesar de menos frequente, têm significante impacto na doença renal crônica.

Métodos:: Relato de caso de mulher, 27 anos, com queixa de dor em pododáctilos bilateralmente havia 3 dias, com presença de pequenas áreas necróticas nos locais referidos. Antecedente pessoal de diabetes tipo 1 (há 25 anos), com doença renal crônica, em diálise peritoneal, além de artrite reumatoide. Teve internação hospitalar, que antecedeu o quadro atual, devido à exacerbação da artrite reumatoide, evoluindo com trombo intracardíaco por complicação de cateter venoso, quando iniciou uso de varfarina. A isquemia progrediu para pés com necessidade de amputações bilaterais. Quirodáctilos também foram acometidos. Trombofilias, vasculites, endocardite ou outras fontes emboligênicas foram pesquisadas e descartadas. Anatomopatológico evidenciou: necrose de pele e partes moles superficiais com trombose arterial recente e calcificação medial de Monckeberg. Tratamento foi instituído com bisfosfonato e tiossulfato de sódio, conversão para hemodiálise e substituição de varfarina por heparina não fracionada. Apesar de toda a terapia, a paciente foi a óbito após quatro meses de evolução.

Discussão:: A calcifilaxia é uma rara síndrome de calcificação da microvasculatura que resulta em graves lesões isquêmicas. Tem patogênese relacionada ao distúrbio mineral e ósseo da doença renal crônica combinado com o desbalanço entre promotores e inibidores de calcificação vascular, com particular importância ao antagonismo da vitamina K.

Conclusão:: A estratégia preventiva é fundamental, uma vez que a terapia é complexa e de eficácia pouco validada.

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Conflict of interest statement

CONFLICT OF INTEREST

There are no conflicts of interest in the present study.

Figures

Figure 1
Figure 1. Initial aspect (A - white arrows) and evolution of the lesion in 1 month (B) and in 3 months (C).
Figure 2
Figure 2. Simple X-Rays showing extensive calcification in the arteries (white arrows).
See this image and copyright information in PMC

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