Calciphylaxis Research Articles

Clinical characteristics and treatment modalities in uremic and non uremic calciphylaxis - a dermatological single-center experience

Calciphylaxis (CP) is a serious, potentially life-threatening disease that presents with medial calcification of small-sized vessels and painful ischemic ulcerations. Although calciphylaxis is frequently seen in patients with end-stage kidney disease on dialysis (calcific uremic arteriolopathy, CUA), there are reported cases of nonuremic calciphylaxis (NUC), which often remain undiagnosed. We conducted a retrospective chart review at our dermatological hospital and evaluated data concerning the epidemiology, comorbidities, medication, laboratory abnormalities, and therapeutic approaches of 60 patients diagnosed with calciphylaxis between 01/2012 and 12/2022. We identified 21 patients diagnosed with NUC and 39 with kidney disease. The predilection sites of skin lesions were the lower legs in 88% (n = 53), followed by the thigh and gluteal regions in 7% (n = 4). Significant differences were identified in comorbidities, such as atrial fibrillation (p < 0.001) and hyperparathyroidism (p < 0.01) accounting for CUA patients. Medication with vitamin K antagonists (p < 0.001), phosphate binders (p < 0.001), and loop diuretics (p < 0.01) was found to be associated with the onset of calciphylaxis. Hyperphosphatemia (p < 0.001), increased parathyroid hormone (p < 0.01) and triglyceride levels (p < 0.01), hypoalbuminemia (p < 0.01) and decreased hemoglobin values (p < 0.001) in the CUA cohort were significantly different from those in the NUC group. All patients with CUA received systemic medication. In contrast, only 38% (n = 8) of patients with NUC received systemic treatment. Striking discrepancies in the treatment of both cohorts were detected. In particular, NUC remains a disease pattern that is still poorly understood and differs from CUA in several important parameters.

Calciphylaxis in Hemodialysis

Calciphylaxis (CP) or uremic calcific arterial disease (CUA) is a rare, potentially fatal calcific vasculopathy characterized by calcific and thrombotic occlusion of the vessels of the subcutaneous and dermis leading to extremely painful necrotic lesions. It mainly affects patients with end-stage kidney disease (ESKD) and under long time dialysis. The only therapeutic option is represented by intravenous sodium thiosulfate. Currently, clear guidelines are lacking. We have had a good therapeutic response with doses of sodium thiosulfate in association with multidisciplinary management of the patient (vulnologist, dermatologist, nephrologist, dietitian, and cardiologist). There is limited literature on the use of DOAC therapy as a successful alternative to warfarin in patients on dialysis with calciphylaxis. The left atrial appendage closure could represent an important alternative to dicumarolics in patients with atrial fibrillation with calciphylaxis. A new perspective for the treatment of this disease is SNF472 a selective inhibitor of vascular calcification.

Diffuse dermal angiomatosis associated with calciphylaxis: A 5-year retrospective institutional review.

Although diffuse dermal angiomatosis (DDA), a rare acquired reactive cutaneous vascular disorder, has been previously reported in association with calciphylaxis (CP), the clinical significance of this relationship has not yet been elucidated. A total of 24 cases of CP diagnosed from 2013 to 2018 were retrospectively reviewed for the presence of associated DDA. Pertinent clinical information for each patient was also collected, and statistical analysis was performed using multivariable logistic regression, Student t test and Fisher exact test. African American race and comorbid congestive heart failure were the only variables that demonstrated independent, statistically significant association with the presence of DDA. End-stage renal failure, diabetes mellitus, immunosuppressive and hypercoagulable states, arrhythmia, body mass index, hypertension, coronary artery disease, patient age, duration of CP symptoms, gender, time interval from biopsy to death, anticoagulation therapy and sodium thiosulfate administration at the time of biopsy did not demonstrate a statistically significant association with DDA. DDA does not appear to be associated with disease severity or prognosis in cases of CP; however, in our population CP with concurrent DDA was more prevalent in African Americans and individuals with congestive heart failure.

The Effectiveness of Topical Cerium Nitrate-Silver Sulfadiazine Application on Overall Outcome in Patients with Calciphylaxis

Background: Calciphylaxis (CPX) is a rare and life-threatening disease characterized by vascular calcification and development of painful and necrotizing skin lesions with a challenging management. Mechanisms of CPX are complex and include an imbalance between vascular calcification promoters and inhibitors, and frequently vitamin K deficiency. Objectives: To describe the various presentations and identify predictive factors of death in patients with CPX. Methods: In this multicenter retrospective study, we included 71 CPX patients followed in South-West France (n = 26) and in French Polynesia (n = 45), and who all received sodium thiosulfate (25 g thrice weekly for a median of 61 days). Results: Characteristics at presentation significantly differed between metropolitan and Polynesian French patients. Polynesians were less frequently on regular dialysis at the onset of CPX, had a higher incidence of diabetes mellitus and obesity, more disturbances of calcium-phosphorus metabolism, and received vitamin K antagonists less frequently than patients from South-West France. Despite intensive management, the 1-year mortality rate was 66% and median time to death was 200 days (IQR, 40; 514). The number of body areas involved (i.e., three: OR 2.70 [1.09; 6.65], p = 0.031; four: OR 8.79 [1.54; 50.29], p = 0.015) was the only predictive factor for death, whereas application of topical cerium nitrate-silver sulfadiazine was protective (OR 0.44 [0.20; 0.99], p = 0.046). Surgical debridement, hyperbaric oxygenation therapy, and geographical origin were not associated with overall outcomes. Conclusions: Cerium nitrate may lead to vascular decalcification and chelation of reactive oxygen species, and prevent infection. Cerium nitrate-silver sulfadiazine was associated with better outcomes and should be tested in a prospective comparative trial in CPX patients.

Calciphylaxis: Challenges in the diagnosis and management.

Calciphylaxis: Challenges in the diagnosis and management.

DOACs least harmful for patients with calciphylaxis on hemodialysis needing anticoagulation

DOACs least harmful for patients with calciphylaxis on hemodialysis needing anticoagulation

Calciphylaxis Masquerading as Warfarin Induced Skin Necrosis

Calciphylaxis is rare complication of end stage renal disease on hemodialysis. Calciphylaxis closely mimics warfarin induced skin necrosis in dialysis patients who are being started on coumadin thus posing a diagnostic challenge. Skin biopsy may be required in such cases to confirm the diagnosis.

Benefits of intralesional injections of sodium thiosulfate in the treatment of calciphylaxis.

Calciphylaxis (CPX) is a rare calcifying thrombotic vasculopathy responsible for painful necrotic ulcers, with a high mortality rate, and its management is often difficult. Recently, intravenous infusions of sodium thiosulfate (STS) have shown efficacy. The aim of this study was to assess the efficacy of intralesional STS (IL STS) in four patients. Our aim was to assess the efficacy of IL STS in a prospective mono-centric open study that included four patients with a biopsy-proven cutaneous CPX. Four women (55-84 years old, mean age: 71·2 years) with a uremic (n = 1) or non-uremic CPX (n = 3) and primary hyperparathyroidism induced by teriparatide or after the initiation of oral anti-vitamin K were treated by IL STS (250 mg/ml). The injections were performed around the ulcers, on the active borders, once or twice a week and then at 1-2 weeks intervals. The injected quantity varied from 1·5 to 15 ml. Pain usually improved after two series of injections. Clinical response was visible after 2 weeks. Three patients (75%) healed completely or almost completely. A failure was observed in the last patient who also had lower limb arteriopathy. The main side effect was the pain during injections. IL STS is an interesting alternative therapeutic option in the management of CPX necrotic ulcers with limited side effects. Larger studies are warranted to precisely define its place, its administration procedure and the patients who could benefit from it.

Calciphylaxis: an overlooked problem of the clinicians

Calciphylaxis: an overlooked problem of the clinicians

Calciphylaxis of the breast with associated diffuse dermal angiomatosis

In 2013, approximately 660,000 Americans had end stage renal disease (ESRD), requiring either dialysis or transplantation. Up to 5% of dialysis dependent patients may develop calcific uremic arteriolopathy or calciphylaxis (CPX) with vascular medial calcification of arterioles and subcutaneous capillaries with superimposed thrombotic occlusion. Risk factors other than chronic kidney disease include: female gender, diabetes mellitus, liver disease, warfarin, obesity, and hypercoagulability. CPX usually affects adipose rich areas on the trunk and proximal extremities, however an increasing number of reports document breast involvement. We report a case of unilateral breast CPX with a protracted clinical course and superimposed diffuse dermal angiomatosis (DDA). These two unusual cutaneous vaso-occlusive conditions have rarely been reported simultaneously. We review the literature and discuss the relationship and predisposing factors between these two conditions. We believe our case suggests that DDA could be precipitated by CPX secondary to calcific vaso-occlusion.

Calciphylaxis: A Large Case Series

Calciphylaxis (also known as calcific uremic arteriolopathy, because it is seen mostly in patients with end-stage renal disease [ESRD]) is an uncommon

Non-Uremic Calcific Arteriolopathy (Calciphylaxis) in Relapsed/Refractory Hodgkin's Lymphoma: A Previously Unreported Association

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La calciphylaxie chez le patient dialysé : la reconnaître pour la traiter aussitôt que possible

Calciphylaxis (CPX) or calcific uraemic arteriolopathy is a rare life-threatening complication, affecting mainly dialysis patients. The condition is characterized by calcifications and thrombosis of the small cutaneous vessels and small vessels in the fat tissue, resulting in the development of necrotizing and non-healing ulcers. The development of these lesions leads to poor outcomes owing to infectious complications and some frequently associated unfavourable medical conditions: obesity, diabetes, and peripheral vascular disease. We report the case of six patients with different clinical forms of CPX in the past 10 years with favourable outcomes observed in five of the six patients. The diagnosis was based on clinical presentation: bilateral and hyperalgesic necrotic lesions along with a history of mineral metabolism disorder or warfarin use. The therapeutic strategy included the following: daily dialysis, hyperbaric oxygen therapy, treatment of limb artery stenosis, maintenance of the optimal haemodynamic stability, delivery of cutaneous care, administration of analgesics and antibiotics, warfarin and calcium cessation, and additional therapy with cinacalcet or parathyroidectomy and therapy with bisphosphonates or sodium thiosulphate. Healing was observed in five out of six CPX patients by using this strategy that should be rapidly employed in order to decrease the necrotizing areas that result in poor outcomes. Prevention includes identification of at-risk patients in order to optimize the treatment of the identified risk factors for CPX.

Diffuse Dermal Angiomatosis Associated With Calciphylaxis

Diffuse dermal angiomatosis (DDA) is a reactive proliferation of vascular channels within the dermis often associated with atherosclerosis. Based on our observation of a case of calciphylaxis (CP) with extensive DDA, we investigated a new possible association and incidence of DDA in patients with CP. These 2 rare conditions had not been reported previously in the same patient. In a retrospective review of skin biopsies taken between 1988 and 2006, 11 patients with histologically proven CP were identified and the medical records were reviewed. Two cases were excluded due to inadequate specimens for a thorough histologic evaluation. Nine patients with large necrotic plaques/ulcers were included in the study. Associated diseases were end-stage renal insufficiency (n = 7), parathyroidectomy for hyperparathyroidism (n = 3), thromboembolic events (n = 3), hypertension (n = 3), and diabetes mellitus (n = 2). Histologically, all cases had some degree of diffuse dermal proliferation of vascular channels with interstitial expression of CD31, as well as subcutaneous fat necrosis and calcification with medial vascular calcification. The extent of DDA did not correlate with the gravity or severity of disease. Based on our observation, DDA is a common histological finding encountered in the dermis adjacent to necrotizing ulcers in patients with CP.

175: Calciphylaxis Induced by Paricalcitol

175: Calciphylaxis Induced by Paricalcitol

Treating distal calciphylaxis with therapy associated with sevelamer and bisphosphonates

Treating distal calciphylaxis with therapy associated with sevelamer and bisphosphonates

A New Medical Treatment for Calciphylaxis

Calciphylaxis, also known as calcific uremic arteriolopathy, is a rare but serious complication of end-stage renal disease, marked by vascular

Vascular injury and mortality in renal osteodystrophy

Cardiovascular disturbance occupied 43.7% on causes of death in dialysis patients. It was confirmed that their deaths were, at least partially, caused by accelerated vascular damage which originates from renal osteodystrophy. This review describes calciphylaxis with very poor prognosis, and advanced calcification in blood vessels and soft tissues in dialysis patients. The relation of coronary artery calcification, of which the quantitative evaluation recently became possible by ultra-first CT, is outlined with special concern to their effects on the mortality.

Calciphylaxis -- Recent Reports on Managing This Life-Threatening Dermatosis

Many widely read journals have recently covered calciphylaxis. W.G. Goodman, among others, prefers the designation calcific uremic arteriolopathy

Calcific Small Vessel Ischemic Disease (Calciphylaxis) in Dialysis Patients

Calcific Small Vessel Ischemic Disease (Calciphylaxis) in Dialysis Patients