A Fatal Case of Calciphylaxis in a Patient with Systemic Lupus Erythematosus and Normal Renal Function

Pek, Elisabeth Anna; Joseph, Pamela L; Habeeb, Ayman Saeed Al; Albert, Lori

doi:10.3899/jrheum.150819

Cited by 4 publications

(4 citation statements)

References 9 publications

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“…Non-uremic calciphylaxis has also been reported in patients with other autoimmune conditions such as RA [ 137 , 138 ], SLE [ 139 , 140 , 141 ], and the concomitance of multiple rheumatologic disease (i.e., SLE, secondary Sjogren’s syndrome, and myasthenia gravis) [ 142 ]. Some proposed or speculated additional mechanisms leading to the development of non-uremic calciphylaxis are D hypovitaminosis and secondary hyperparathyroidism, often associated with mild renal disjunction occurring in patients with autoimmune diseases, and the mild hypercoagulable state induced by the development of anti-protein S antibodies in patients with RA [ 142 ].…”

Section: Calciphylaxis and Autoimmune Diseasesmentioning

confidence: 99%

Calcinosis Cutis and Calciphylaxis in Autoimmune Connective Tissue Diseases

et al. 2023

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Calcinosis represents a severe complication of several autoimmune disorders. Soft-tissue calcifications have been classified into five major types: dystrophic, metastatic, idiopathic, iatrogenic, and calciphylaxis. Autoimmune diseases are usually associated with dystrophic calcifications, including calcinosis cutis, occurring in damaged or devitalized tissues in the presence of normal serum levels of calcium and phosphate. In particular, calcinosis cutis has been described in dermatomyositis, polymyositis, juvenile dermatomyositis, systemic sclerosis, systemic lupus erythematosus, primary Sjögren’s syndrome, overlap syndrome, mixed connective tissue disease, and rheumatoid arthritis. Calciphylaxis, a severe and life-threatening syndrome presenting with vascular calcifications and thrombosis, has also been associated with some autoimmune conditions. Due to the potentially disabling character of calcinosis cutis and calciphylaxis, physicians’ awareness about the clinical presentation and management of these diseases should be increased to select the most appropriate treatment option and avoid long-term complications. In this review, we aim to analyze the clinical features of calcinosis cutis and calciphylaxis associated with autoimmune diseases, and the main treatment strategies evaluated up to now for treating this potentially disabling disease.

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Section: Calciphylaxis and Autoimmune Diseasesmentioning

confidence: 99%

Calcinosis Cutis and Calciphylaxis in Autoimmune Connective Tissue Diseases

et al. 2023

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“…Interestingly, studies have found that the total amount and density of perivascular adipose tissue in women with SLE are related to vascular calcification, 64 , 65 which may provide a basis to explain why calciphylaxis often occurs in regions with high‐fat content. It is well known that patients with connective tissue diseases often use corticosteroids to control disease activity 66 or warfarin for anticoagulation, 57 both of which can induce calciphylaxis. Patients with SLE or antiphospholipid syndrome often have different pathological types of renal involvement, 67 which may accelerate the progression of calciphylaxis.…”

Section: Calciphylaxis Associated With Connective Tissue Diseasesmentioning

confidence: 99%

“…We searched PubMed and found 28 connective tissue disease‐related calciphylaxis reports published as of August 2022, in which most of the patients were middle‐aged women. Among the connective tissue diseases, systemic lupus erythematosus (SLE) is most often associated with calciphylaxis (approximately 54%), 3,57 followed by calciphylaxis with rheumatoid arthritis (approximately 29%) 12,58,59 . There are also cases of calciphylaxis with giant cell arteritis, antiphospholipid syndrome, and psoriatic arthritis 60–62 .…”

Section: Calciphylaxis Associated With Connective Tissue Diseasesmentioning

confidence: 99%

Calciphylaxis and its co‐occurrence with connective tissue diseases

Zhou

Chen

Yin

et al. 2022

International Wound Journal

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Calciphylaxis, also known as calcific uremic arteriopathy, is a rare calcification syndrome that presents as ischemic skin necrosis and severe pain. It has a high mortality rate and is characterised by calcification of the small and medium arteries and micro‐thrombosis. Calciphylaxis mainly occurs in patients with end‐stage renal disease. In recent years, there have been an increasing number of cases of calciphylaxis associated with connective tissue diseases. Given the absence of clear diagnostic criteria for calciphylaxis thus far, an early diagnosis is crucial for designing an effective multidisciplinary treatment plan. In this article, we review the research progress on calciphylaxis and describe its characteristics in the context of connective tissue diseases.

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“…It leads to thrombosis, ischemia, and cutaneous necrosis 1 . Calciphylaxis is typically seen in the setting of end-stage renal disease, but it has been increasingly reported in patients with preserved renal function and patients with systemic lupus erythematosus (SLE) 2, 3, 4, 5, 6. We report an unusual, highly aggressive, and rapidly progressive case of fatal nonuremic calciphylaxis with atypical clinical and histopathologic features in a patient with SLE.…”

Section: Introductionmentioning

confidence: 99%