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Multiple extremity necrosis in fatal calciphylaxis: Case report
Diego Ennes Gonzalez1, Renato Demarchi Foresto1, Ana Luiza Santos Maldonado1
1Universidade Federal de São Paulo (UNIFESP), Escola Paulista de Medicina (EPM), Hospital do Rim, São Paulo, SP, Brasil.
Insights
Calciphylaxis, a rare syndrome causing severe ischemic injuries, impacts chronic kidney disease patients. This case highlights the challenges in managing this condition, emphasizing the need for preventive strategies due to limited therapeutic effectiveness.
Area of Science:
- Nephrology
- Vascular Medicine
- Dermatology
Background:
- Vascular calcification significantly impacts cardiovascular health and is a serious concern in chronic kidney disease (CKD).
- Calciphylaxis, a rare but severe microvascular complication, presents unique challenges in CKD patients.
- Understanding the pathogenesis of calciphylaxis is crucial for developing effective management strategies.
Observation:
- A 27-year-old female with a 25-year history of type 1 diabetes and CKD on peritoneal dialysis developed painful necrotic lesions on her toes.
- The patient had a history of rheumatoid arthritis and recent complications including intracardiac thrombus, necessitating warfarin therapy.
- Progressive ischemia led to bilateral lower extremity amputations and affected her hands, with investigations ruling out common embolic sources.
Findings:
- Pathology confirmed skin necrosis with arterial thrombosis and Monckeberg's medial calcification, indicative of calciphylaxis.
- Treatment included bisphosphonate, sodium thiosulfate, conversion to hemodialysis, and heparin, but the patient ultimately succumbed to the condition.
- The case underscores the complex interplay of mineral and bone disorders in CKD, vitamin K antagonism, and vascular calcification in calciphylaxis.
Implications:
- Calciphylaxis management is complex, with current therapies offering limited validated effectiveness.
- Preventive strategies are paramount for improving outcomes in patients with CKD and calciphylaxis.
- This case highlights the critical need for further research into the pathogenesis and treatment of calciphylaxis.
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.
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