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Original Investigation |

Identification of Clinical Features and Autoantibodies Associated With Calcinosis in Dermatomyositis

Antonia Valenzuela, MD, MS1; Lorinda Chung, MD, MS1,2; Livia Casciola-Rosen, PhD3; David Fiorentino, MD, PhD1,2
[+] Author Affiliations
1Division of Immunology and Rheumatology, Stanford University School of Medicine, Stanford, California
2Department of Dermatology, Stanford University School of Medicine, Stanford, California
3Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, Maryland
JAMA Dermatol. 2014;150(7):724-729. doi:10.1001/jamadermatol.2013.10416.
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Importance  Prior studies have estimated that up to 20% of adults with dermatomyositis (DM) have calcinosis, which can lead to significant morbidity. Identification of risk factors may provide a better understanding of the pathogenesis and ultimately therapy for this difficult clinical problem. Risk factors for calcinosis in adults with DM have not been extensively studied.

Objectives  To determine the prevalence of calcinosis and to identify associated clinical features in a cohort of extensively phenotyped adults with DM.

Design, Setting, and Participants  A cross-sectional study of 126 patients diagnosed as having DM from January 1, 2006, through January 1, 2013, was performed. Patients were adults (≥18 years of age) attending the Stanford University Medical Center clinic.

Main Outcomes and Measures  Calcinosis, defined as the presence of calcium deposition in the skin and subcutaneous tissues on physical examination.

Results  Fourteen patients (11.1%) had calcinosis, with the extremities most commonly involved. Patients with vs those without calcinosis had a longer disease duration (median, 6.9 years; range, 2.4–18.1; vs median, 3.9 years; range, 0.2-19.2 years; P = .003) and more fingertip ulcers (50.0% vs 9.3%, P < .001). An association between calcinosis and both interstitial lung disease and anti–MDA-5 autoantibodies was identified, but this association did not persist in multivariate models that adjusted for fingertip ulcers. Fingertip ulcers and disease duration were strongly associated with calcinosis in all multivariate models, independent of the underlying autoantibody present. Autoantibodies to NXP-2 were associated with calcinosis (odds ratio, 15.52; 95% CI, 2.01-119.90), whereas anti–transcriptional intermediary factor 1-γ antibodies were protective (odds ratio, 0.2; 95% CI, 0.01-0.99) in multivariate analyses that adjusted for fingertip ulcers and other covariates.

Conclusions and Relevance  Calcinosis was a relatively uncommon clinical feature in our cohort of adults with DM. Our data suggest that calcinosis is positively associated with longer disease duration, fingertip ulcers, and NXP-2 autoantibodies and negatively associated with transcriptional intermediary factor 1-γ antibodies. A common vascular mechanism may underlie the development of both calcinosis and fingertip ulcers in patients with DM.

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Examples of Calcinosis in Adults With Dermatomyositis

A, Multiple, grouped white papules on the elbow with associated scarring and scale crust. B, Large, multiloculated hard tumors of calcium with characteristic skin retraction extending along the axilla.

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“Dermatomyositis” Is No Longer Enough
Posted on October 3, 2014
Richard D. Sontheimer M.D.
University of Utah School of Medicine
Conflict of Interest: None Declared
A casual reader perusing the July, 2014 issue of JAMA Dermatology might be confused by the title of the report by Valenzuela and coworkers (“Identification of Clinical Features and Autoantibodies Associated with Calcinosis in Dermatomyositis) (1). Upon scanning from the title to the summary catch phrase in the Conclusion and Relevance section of the abstract (\"Our data suggest...\"), such a reader might be led to over generalize the results of this report. The potentially confounding issue here is the fact that this study examined only two of the four major subphenotypes of dermatomyositis – adult-onset classic dermatomyositis (AOCD) and adult-onset clinically-amyopathic dermatomyositis (AOCAD) (2). By using the unmodified root designation \"dermatomyositis\" in the title of their report, the casual reader might presume that these data apply to all dermatomyositis subphenotypes including those with the juvenile-onset classic dermatomyositis (JOCD) and juvenile-onset clinically-amyopathic dermatomyositis subphenotypes (JOCADM) (see accompanying Figure). It is widely recognized that clinically important differences exist between AOCD and JOCD with respect to risk for interstitial lung disease and occult internal malignancy. Likewise, cutaneous calcinosis is a more prevalent and potentially more serious clinical problem in JOCD compared to AOCD. In addition, reviews of the published literature in 2006 (3) and 2007 (4) suggested that cutaneous calcinosis is more prevalent in JOCAD than AOCAD. These analyses also found that interstitial lung disease was more prevalent in AOCAD than JOCAD. Since 1990, there have been 287 PubMed cited publications characterizing the amyopathic dermatomyositis subphenotype (data reviewed in (5)). Population-based epidemiologic studies have suggested that amyopathic dermatomyositis might account for 20% of the total population of dermatomyositis patients. A subgroup of AOCAD patients identified by the presence of circulating CADM-140/MDA-5 autoantibodies is at increased risk for developing potentially fatal interstitial lung disease. Therefore, the lingering resistance by some to accept the clinical concept of amyopathic dermatomyositis is becoming more difficult to defend. Valenzuela et al are to be congratulated for providing needed data to extend our understanding of the clinical significance of cutaneous calcinosis in adult-onset dermatomyositis patients. The point here is that it should no longer be enough to report clinical association data under the unmodified root designation \"dermatomyositis.\"REFERENCES1. Valenzuela A, Chung L, Casciola-Rosen L, Fiorentino D. Identification of Clinical Features and Autoantibodies Associated with Calcinosis in Dermatomyositis: JAMA Derm 2014:150(7)724-7292. Sontheimer RD: Cutaneous features of classic dermatomyositis and amyopathic dermatomyositis. Curr Opin Rheumatol. 1999 Nov;11(6):475-82.3. Gerami P, Schope JM, McDonald L, Walling HW, Sontheimer RD. A systematicreview of adult-onset clinically amyopathic dermatomyositis (dermatomyositis sine myositis): a missing link within the spectrum of the idiopathic inflammatorymyopathies. J Am Acad Dermatol. 2006 Apr;54(4):597-613. 4. Gerami P, Walling HW, Lewis J, Doughty L, Sontheimer RD. A systematic review of juvenile-onset clinically amyopathic dermatomyositis. Br J Dermatol. 2007Oct;157(4):637-44. 5. Ghazi E, Sontheimer RD, Werth VP. The importance of including amyopathic dermatomyositis in the idiopathic inflammatory myositis spectrum. Clin Exp Rheumatol. 2013 Jan-Feb;31(1):128-34. FIGURE LEGENDGraphic illustration of the modern major subphenotypes of dermatomyositis.
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