Calcinosis is associated with digital ischaemia in systemic sclerosis—a longitudinal study

Baron, Murray; Pope, Janet; Robinson, David; Jones, Niall; Khalidi, Nader; Docherty, Peter; Kamińska, Elżbieta; Masetto, Ariel; Sutton, Evelyn; Mathieu, Jean‐Pierre; Ligier, Sophie; Grodzicky, Tamara; LeClercq, Sharon; Thorne, Carter; Gyger, Geneviève; Smith, D. P.; Fortin, Paul R.; Larché, Maggie; Abu-Hakima, Maysan; Rodríguez‐Reyna, Tatiana Sofía; Cabral-Castañeda, Antonio; Fritzler, Marvin J.; Wang, Mianbo; Hudson, Marie

doi:10.1093/rheumatology/kew313

Cited by 59 publications

(52 citation statements)

References 27 publications

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“…Only prospective longitudinal studies with long-term followup will allow us to draw conclusions regarding any common causal factors of micro-and macrovascular involvement. Two recent studies have highlighted the associations of calcinosis with digital ischemia and late NFC pattern in SSc (2,3). Morardet et al (2) have also reported an association between acroosteolysis and late NFC pattern.…”

Section: Discussionmentioning

confidence: 98%

“…There is a lack of therapeutic measures to tackle these digital manifestations of the disease, and a better characterization of the mechanisms underlying acroosteolysis and calcinosis is therefore needed. Recent cross-sectional studies have reported an association of these digital manifestations with Cutolo's late capillaroscopic pattern, defined by severe capillary loss and proliferative neovascularization, supporting a vascular etiology of both acroosteolysis and calcinosis (2,3).…”

Section: Introductionmentioning

confidence: 92%

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Combination of Capillaroscopic and Ultrasonographic Evaluations in Systemic Sclerosis: Results of a Cross‐Sectional Study

Lescoat

Coiffier

Carlan

et al. 2018

Arthritis Care & Research

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Section: Discussionmentioning

confidence: 98%

Section: Introductionmentioning

confidence: 92%

Combination of Capillaroscopic and Ultrasonographic Evaluations in Systemic Sclerosis: Results of a Cross‐Sectional Study

Lescoat

Coiffier

Carlan

et al. 2018

Arthritis Care & Research

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“…It is posited that tissue ischemia by vasculopathy may contribute to the development of cutaneous calcinosis . However, the pathogenesis of spinal calcinosis in SSc is not well‐known.…”

Section: Discussionmentioning

confidence: 99%

“…It is posited that tissue ischemia by vasculopathy may contribute to the development of cutaneous calcinosis. [7][8][9] However, the pathogenesis of spinal calcinosis in SSc is not wellknown. Ogawa et al 15 reported that Raynaud's phenomenon and digital ulcers were significantly prevalent in SSc patients with spinal calcinosis.…”

Section: Discussionmentioning

confidence: 99%

“…Subcutaneous small nodules occurring in the fingers are classified as “dystrophic calcification”, and large subcutaneous calcium deposits near the joints or pressure areas are called “tumoral calcification.” Although the etiology of calcinosis in SSc is not well known, it is hypothesized that tissue ischemia by vasculopathy may contribute to the development of calcinosis. Baron et al . reported that 23% of SSc patients had cutaneous calcinosis, which was associated with digital ischemia, ischemia severity, capillary dropout, anticentromere antibody (Ab) and anti‐RNA polymerase III Ab.…”

Section: Introductionmentioning

confidence: 99%

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Demographic and clinical characteristics of spinal calcinosis in systemic sclerosis: Possible association with peripheral angiopathy

Motegi

Sekiguchi

Yonemoto

et al. 2018

The Journal of Dermatology

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The objective was to evaluate the demographic and clinical characteristics of systemic sclerosis (SSc) patients with spinal calcinosis. Paraspinal and intraspinal calcinosis was assessed blindly by orthopedic surgeons specializing in spinal diseases using chest high‐resolution computed tomography (CT) that was performed for the screening and prospecting of interstitial lung disease in 159 Japanese SSc patients. Among these patients, we identified 27 (17%) with spinal calcinosis, and the most common site was cervical level at 77.8% (21/27). The frequency of spinal calcinosis in the late stage was higher than in the early stage (44.4% vs 29.6%). Multiple calcinosis was identified in 18.5% (5/27). The frequency of paraspinal calcinosis only was 59.3%, intraspinal calcinosis only 18.5%, and both intraspinal and paraspinal calcinosis 22.2%. Among SSc patients, 4.4% (7/159) had CT‐based evidence of spinal cord compression. Among cases with spinal cord compression, only one had neurological symptoms, and surgical removal improved the symptoms. The other six SSc patients with spinal calcinosis (3.8% of 159) had no symptoms. Male sex (29.6%) and severe peripheral vasculopathy such as digital ulcers (55.6%) and acro‐osteolysis (33.3%) were significantly more frequent in the SSc patients with spinal calcinosis than in the SSc patients without spinal calcinosis (10.6%, 32.6% and 14.4%, respectively). Our results suggest that severe peripheral vasculopathy may be associated with the development of spinal calcinosis. Because SSc patients are prone to spinal calcinosis, when SSc patients claim symptoms such as pain, numbness and movement disorder of the extremities, spinal calcinosis is a complication that should be taken into consideration.

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Clinical Associations of Degos-Like Lesions in Patients With Systemic Sclerosis

Song

Lewis

et al. 2023

JAMA Dermatol

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ImportanceDegos-like lesions are cutaneous manifestations of a small-vessel vasculopathy that appear as atrophic, porcelain-white papules with red, telangiectatic borders. No study has adequately examined Degos-like lesions in patients with systemic sclerosis (SSc).ObjectiveTo characterize the serologic, cutaneous, and internal organ manifestations associated with Degos-like lesions in a large cohort of patients with SSc.Design, Settings, and ParticipantsThis retrospective cohort study involved adult patients with SSc who were seen at Stanford Rheumatologic Dermatology Clinic between January 1, 1998, and December 31, 2018. Participants fulfilled the 2013 classification criteria for SSc. Data analysis was conducted from February 1 to June 1, 2019.Main Outcomes and MeasuresData on demographic characteristics; autoantibody status; clinical characteristics, including cutaneous and systemic manifestations of SSc; and presence of Degos-like lesions were collected.ResultsThe cohort comprised 506 patients with SSc (447 females [88.3%]; mean [SD] age at first non–Raynaud disease symptoms, 46.1 [15.2] years). Twenty-seven patients (5.3%) had Degos-like lesions, of whom 24 (89.0%) had lesions affecting the fingers. Patients with Degos-like lesions were more likely to have diffuse cutaneous SSc compared with patients without lesions (15 [55.6%] vs 181 [37.8%]; P = .04). Degos-like lesions were also associated with acro-osteolysis (10 [37.0%] vs 62 [12.9%]; P &lt; .01), digital ulcers (15 [55.6%] vs 173 [36.1%]; P = .04), and calcinosis (15 [55.6%] vs 115 [24.0%]; P &lt; .01). While Degos-like lesions were not associated with internal organ manifestations, such as scleroderma renal crisis, interstitial lung disease, or pulmonary arterial hypertension, there was P &lt; .10 for the association with gastric antral vascular ectasia.Conclusions and RelevanceResults of this study suggest an association of Degos-like lesions with diffuse cutaneous SSc and other cutaneous manifestations of vasculopathy, including acro-osteolysis, calcinosis, and digital ulcers. A prospective longitudinal study is warranted to examine the onset of Degos-like lesions and to elucidate whether these lesions play a role in SSc.

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Calcinosis is associated with digital ischaemia in systemic sclerosis—a longitudinal study

Abstract: Ischaemia may play a role in the development of calcinosis in SSc.

Cited by 59 publications

References 27 publications

Combination of Capillaroscopic and Ultrasonographic Evaluations in Systemic Sclerosis: Results of a Cross‐Sectional Study

Combination of Capillaroscopic and Ultrasonographic Evaluations in Systemic Sclerosis: Results of a Cross‐Sectional Study

Demographic and clinical characteristics of spinal calcinosis in systemic sclerosis: Possible association with peripheral angiopathy

Clinical Associations of Degos-Like Lesions in Patients With Systemic Sclerosis

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