A Case of Early Calciphylaxis Diagnosed by Bone Scan.
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ABSTRACT:
SUBMITTER: Di J
PROVIDER: S-EPMC7103050 | biostudies-literature | 2020
REPOSITORIES: biostudies-literature
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A Case of Early Calciphylaxis Diagnosed by Bone Scan.
Di Jia J Liu Yuqiu Y Wang Dongyan D Yang Min M
Case reports in medicine 20200317
Calcium uremic aortic disease (calciphylaxis) has long been considered as a rare, life-threatening small vessel disease. The diagnosis of calciphylaxis depends mainly on clinical symptoms and high risk factors, and skin biopsy can be used to confirm the diagnosis. However, noninvasive testing methods are still the focus of exploration currently. There is increasing evidence that bone scintigraphy is helpful in the diagnosis of calciphylaxis, especially for assessing the involvement of muscles an ...[more]
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Project description:RationaleThe recognition of calciphylaxis often eludes practitioners because of its multiple ambiguous presentations. It classically targets areas of the body dense with adipose tissue. A heightened suspicion for the disorder is therefore required in the case of penile calciphylaxis, given its unconventional location. The diagnosis of calciphylaxis is also challenging as the gold standard for diagnosis is biopsy which can often yield equivocal results. Unfortunately, in penile calciphylaxis, the utility of biopsies is further debated due to their potential to precipitate new lesions and their decreased sensitivity due to the limited depth of tissue that can be sampled. For these reasons, it is important that practitioners recognize other accessible and accurate investigative tools which can aid in their diagnosis.Presenting concerns of the patientWe present the case of a 49-year-old man who presented to the emergency room with penile pain in the context of known chronic kidney disease secondary to diabetic nephropathy. The pain had been present for about a week, was exquisitely tender, and was initially associated with a faint violaceous lesion. This gentleman had just recently initiated peritoneal dialysis and had no other lesions on his body.DiagnosisHis pain was determined by ultrasound and plain radiograph to be secondary to calciphylaxis after two biopsies were nondiagnostic.InterventionsThe patient had already made changes to his diet to reduce phosphate and calcium intake, and had been on phosphate-lowering therapy with both calcium and phosphate being within their respective target range. Following his diagnosis, this patient was promptly converted from peritoneal dialysis to hemodialysis with sodium thiosulphate and initiated hyperbaric oxygen therapy. This patient continues to be followed by nephrology and urology specialists.
| S-EPMC8317242 | biostudies-literature
Project description:IntroductionThe 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.MethodsCase 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.DiscussionCalciphylaxis 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.ConclusionThe preventive strategy is fundamental, since the therapy is complex with poorly validated effectiveness.
| S-EPMC8257280 | biostudies-literature
Project description:Calciphylaxis remains a poorly understood vascular calcification disorder with predilection for patients with end-stage renal disease (ESRD). Recent data from large patient registries and databases have begun to provide information regarding incidence, risk factors, and outcomes in patients with calciphylaxis.The most recent estimate places the incidence of calciphylaxis at 3.5 new cases/1000 patient-years among the patients with ESRD on chronic hemodialysis. It is possible that misdiagnosis or subclinical disease may attribute to lower than the true incidence. There is a suggestion that the incidence is higher in peritoneal dialysis patients compared with patients with hemodialysis. Recent studies have identified a number of risk factors and point to the effects of vitamin K deficiency mediated impairment in Matrix Gla Protein carboxylation as one of the likely pathogenic mechanisms. The outcomes in calciphylaxis patients remain poor with mortality approaching 30% at 6 months and 50% at 12 months.The present review describes recent literature in the field of calciphylaxis. Calciphylaxis registries and specimen biorepositories promise to provide insights into the pathogenesis of calciphylaxis and will pave the way for much needed clinical trials.
| S-EPMC5907931 | biostudies-literature
Project description:Primary splenic angiosarcoma carries a poor prognosis and is among the rarest forms of malignancy. An overwhelming majority of patients with splenic angiosarcoma will develop metastases. However, osseous metastatic disease is rare. We present an 83 year old hispanic female who was diagnosed with primary splenic angiosarcoma on bone marrow biopsy performed for a hematologic workup. We highlight key historical, laboratory, imaging, and pathological features of splenic angiosarcoma. The synthesis of both imaging features and clinical history is essential for establishing early diagnosis in these patients.
| S-EPMC6321865 | biostudies-other
Project description:BACKGROUND:The objective of this study was to investigate the role of bone morphogenetic protein (BMP) signal transduction in the pathogenesis of calciphylaxis. METHODS:Skin biopsy specimens were obtained from 18 patients with, and 12 patients without, calciphylaxis. Tissue sections were stained with antibodies directed against BMP effector proteins phosphorylated-SMAD (p-SMAD) 1/5/9, inhibitor of DNA 1 (Id1), inhibitor of DNA 3 (Id3), and Runx2. The intensity of staining was scored semi-quantitatively as strong versus weak or absent. RESULTS:Of the 18 patients with calciphylaxis (mean age: 59 ± 8 years), 9 were women and 15 had end-stage renal disease. Of the 12 control patients (mean age: 57 ± 10 years), 8 were women and 8 had end-stage renal disease. Strong staining for p-SMAD 1/5/9 was detected in blood vessels from all calciphylaxis patients. In 1 patient with calciphylaxis, strong staining for p-SMAD 1/5/9 was detected in a blood vessel that did not have evidence of calcification. Id1 and Id3 immunoreactivity was detected in blood vessels from all 12 patients with calciphylaxis that were tested. Runx2 staining was detected in all 6 patients with calciphylaxis who were tested. p-SMAD 1/5/9 immunoreactivity was weak or absent in blood vessels of 10 of the 12 control samples. CONCLUSIONS:The BMP signal transduction pathway is activated in the cutaneous vasculature of calciphylaxis patients. The ability to detect p-SMAD 1/5/9, Id1, and Id3 in cutaneous vasculature may assist in the diagnosis of calciphylaxis. As BMP signaling inhibitors become available, this pathway may serve as a future therapeutic target for calciphylaxis.
| S-EPMC5763243 | biostudies-literature
Project description:Importance:Classic calciphylaxis associated with renal failure is a life-threatening disease. Warfarin-associated calciphylaxis without renal injury has been described, but whether it is a subset of classic calciphylaxis or a different entity remains unknown. We describe 1 case of warfarin-associated calciphylaxis, present data from 2 others from our institution, and review all cases of warfarin-associated calciphylaxis available in the literature. Our review indicates that warfarin-associated calciphylaxis is clinically and pathophysiologically distinct from classic calciphylaxis. Objective:To review warfarin-associated calciphylaxis and determine its relationship to classic calciphylaxis. Design, Setting, and Participants:We searched MEDLINE and Ovid without language or date restrictions for case reports of calciphylaxis from the inpatient setting using the terms "calciphylaxis and warfarin," "non-uremic calciphylaxis," and "nonuremic calciphylaxis." We defined nonuremic calciphylaxis as a histopathologic diagnosis of calciphylaxis without severe kidney disease (serum creatinine level >3 mg/dL; glomerular filtration rate <15 mL/min; acute kidney injury requiring dialysis; and renal transplantation). Exposures:Each patient had been exposed to warfarin before the onset of calciphylaxis. Main Outcomes and Measures:Patient data were abstracted from published reports. Original patient medical records were requested and reviewed when possible. Results:We identified 18 patients with nonuremic calciphylaxis, 15 from the literature, and 3 from our institution. Patients were predominantly female (15 of 18 [83%]) with ages ranging from 19 to 86 years. Duration of warfarin therapy prior to calciphylaxis onset averaged 32 months. Lesions were usually located below the knees (in 12 of 18 [67%]). No cases reported elevated calcium-phosphate products (0 of 17 [0%]). Calcifications were most often noted in the tunica media (n?=?8 [44%]) or in the vessel lumen and tunica intima (n?=?7 [39%]). The most common treatments included substitution of heparin or low-molecular weight heparin for warfarin (n?=?13 [72%]), intravenous sodium thiosulfate (n?=?9 [50%]), and hyperbaric oxygen (n?=?3 [17%]). The survival rate on hospital discharge was remarkably high, with 15 cases (83%) reporting full recovery and 3 cases ending in death. Conclusions and Relevance:Warfarin-associated calciphylaxis is distinct from classic calciphylaxis in pathogenesis, course, and, particularly, outcome. This finding should influence clinical management of the disease and informs targeted treatment of the disease.
| S-EPMC5703198 | biostudies-literature
Project description:Calciphylaxis is a deadly, painful disease with a 1-year mortality of up to 50%. The disease is commonly associated with patients with end-stage kidney disease (ESKD), but it can manifest in non-uremic patients as well. In patients who are undergoing dialysis, the incidence of calciphylaxis can range from 0.04% to 4%. The progressive arterial calcification seen in calciphylaxis can affect multiple body organs, including the skin, brain, lungs, and muscle. In cutaneous calciphylaxis, painful and non-healing nodules, plaques, and ulcers may appear, increasing morbidity for patients. Diagnosis can be difficult, and the condition can clinically appear similar to other dermatological diseases, especially in non-uremic patients. Currently, skin biopsy with histological analysis is the most reliable method to help diagnose the condition. In certain cases, the use of medical imaging may be helpful. Treatment of pain in this condition can be difficult and should be multimodal and include wound care as well as modification of risk factors. Analgesic options include opioids, nonsteroidal anti-inflammatory drugs (NSAIDs), as well as analgesic options that are targeted for specific patients. There are currently multiple clinical trials underway that are studying targeted therapies for this condition.
| S-EPMC7595979 | biostudies-literature
Project description:Importance:Calciphylaxis is a rare skin disease with high morbidity and mortality that frequently affects patients with renal disease. Hypercoagulable conditions are frequently observed in both patients with calciphylaxis and those with chronic kidney disease (CKD), complicating our understanding of which hypercoagulable conditions are specific to calciphylaxis. Objective:To identify hypercoagulable conditions that are risk factors for developing calciphylaxis while controlling for CKD. Design, Setting, and Participants:This was a case-control study, comparing the hypercoagulability status of patients with calciphylaxis and with renal disease with that of a matched control population at 2 large urban academic hospitals in Boston, Massachusetts. Retrospective medical record review of laboratory values was performed to identify patients with hypercoagulable conditions. Case and control patients were further stratified based on both severity of CKD and warfarin. Patients with a dermatologic diagnosis of calciphylaxis between 2006 and 2014 and concomitant CKD were included as cases (n?=?38). Three controls (n?=?114) per case patient with CKD were included, and were matched by age, sex, and race. Main Outcomes and Measures:The rate of various hypercoagulable states (ie, antithrombin III [ATIII] deficiency, protein C and S deficiency, factor V Leiden mutation, prothrombin gene mutation [G20210A], elevated factor VIII level, lupus anticoagulant, anti-IgG or IgM cardiolipin antibodies, heparin-induced thrombocytopenia antibodies, and elevation of homocysteine) in patients with calciphylaxis compared with their matched controls. Results:Of the calciphylaxis cohort, 28 (58%) were female and 18 (55%) were non-Hispanic white. Among all patients, lupus anticoagulant (13 [48%] positive in cases vs 1 [5%] in controls; P?=?.001), protein C deficiency (9 [50%] vs 1 [8%]; P?=?.02), and combined thrombophilias (18 [62%] vs 10 [31%]; P?=?.02) were found to be significantly associated with calciphylaxis. In a subanalysis of patients with stage 5 CKD, only lupus anticoagulant (12 [53%] vs 9 [0%]; P?=?.01) and combined thrombophilia (15 [63%] vs 1 [8%]; P?=?.004) remained significantly associated with calciphylaxis. In a separate subanalysis of warfarin-unexposed patients, only lupus anticoagulant (7 [50%] vs 1 [6%]; P?=?.01) and protein C deficiency (5 [46%] vs 10 [0%]; P?=?.04) remained significantly associated with calciphylaxis. Conclusions and Relevance:Presence of lupus anticoagulant and combined thrombophilias are risk factors for the development of calciphylaxis in patients with late-stage renal disease. Clinicians should be aware of these associations in patients with impaired kidney function and may consider increased screening and appropriate anticoagulation treatment to reduce the risk of calciphylaxis development.
| S-EPMC5839276 | biostudies-literature
Project description:Calciphylaxis is a clinical syndrome of arteriolar calcification with resultant tissue necrosis, most commonly seen in patients with end-stage renal disease (ESRD) on dialysis. This condition carries a high mortality rate mainly due to infection. Helpful interventions include adequate dialytic therapy, medical management of hyperparathyroidism, hyperphosphatemia, and intravenous sodium thiosulfate therapy.
| S-EPMC5538074 | biostudies-other
Project description:Calciphylaxis is a rare but devastating condition that has continued to challenge the medical community since its early descriptions in the scientific literature many decades ago. It is predominantly seen in patients with chronic kidney failure treated with dialysis (uremic calciphylaxis) but is also described in patients with earlier stages of chronic kidney disease and with normal kidney function. In this review, we discuss the available medical literature regarding risk factors, diagnosis, and treatment of both uremic and nonuremic calciphylaxis. High-quality evidence for the evaluation and management of calciphylaxis is lacking at this time due to its rare incidence and poorly understood pathogenesis and the relative paucity of collaborative research efforts. We hereby provide a summary of recommendations developed by a multidisciplinary team for patients with calciphylaxis.
| S-EPMC4696752 | biostudies-literature