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SAT0469 Calcinosis Cutis in Poly-Dermatomyositis: Clinical and Therapeutic Study
  1. M. Fredi1,
  2. F. Bartoli1,
  3. I. Cavazzana1,
  4. N. Carabellese1,
  5. A. Ceribelli2,
  6. A. Tincani1,
  7. M. Satoh3,
  8. F. Franceschini1
  1. 1Rheumatology and Clinical Immunology Unit, Brescia
  2. 2Humanitas Research Hospital BIOMETRA Department, Rozzano, Italy
  3. 3School of Health Sciences University of Occupational and Environmental Health, Kitakyushu, Japan

Abstract

Background Subcutaneous or soft-tissue calcification is a recognized manifestation of many connective-tissue diseases; however, little is known about the pathogenesis and the treatment of this condition.

Objectives To identify the possible clinical and laboratory predictors of calcinosis by analyzing clinical and immunologic characteristic in a cohort of patients with a diagnosis of Poly-dermatomyositis (PDM).

Methods Seventy-four patients with a diagnosis of PDM referred to our Rheumatology and Immunology Unit were studied: 16 had calcinosis (C+) and 58 did not (C-). Clinical and laboratory data were collected from clinical charts. Statistical analysis was performed using the Chi-squared test and Student t test, p<0.05 is considered statistically significant.

Results The diagnoses in the C+ group were Dermatomyositis (12 patients), Polymyositis (1 patient) and Polymyositis and Systemic Sclerosis (SSc) Overlap syndrome (3 patients).

The mean age at onset in the C+ group was 42.9 years (±20.4) and there was a predominance of females (81% vs 78%).

Calcinosis occurred on average 43.7 months (SD 71.4 months) after diagnosis of connective-tissue disease. The first lesion was single in 11 cases (69%) and multiple in 5 (31%), with a predominant involvement of pelvic girdle (50%), root of higher limbs (25%), hands (25%), forearms and legs (13%). Pain associated to calcinosis was present in 9 cases (56%), while ulceration or superinfection of calcinosis were detected in 7 patients (44%).

Therapies used for calcinosis were: colchicine (56%), diltiazem (44%), IVIg (44%), sodium thiosulfate cream (31%), calcium-channel blockers (19%), warfarin (13%), rituximab (13%), infliximab (13%). A limited improvement was reported: subjective improvement (colchicine,1 case), reduction in pain (IVIg,1) and resolution of rubor overlying calcifications (rituximab,1). Surgical treatment of calcinosis was conducted in 38% of PDM patients.

C+ group distinguishing features compared to C- group are shown in the table. Notably 4 out of 5 anti-PM/Scl (80%) as well as 4 out of 8 (50%) anti-MJ/NXP2 antibodies were found in patients with calcinosis.

Conclusions PDM patients with calcinosis constitute a disease subset characterized by: higher frequency of DM, inflammatory skin involvement as Gottron's papules, and increased frequency of pulmonary involvement (lower DLCO/VA)

Anti-PM/Scl antibodies are more frequent in the C+ group (p<0.007): this reflects the increased frequency of Overlap PM-SSc Syndrome in C+ compared to C- (18.7% vs 9%). Anti-MJ/NXP-2 positivity tend to be more frequent in the C+ group (p=0.06).

No drug treatment is effective in reducing the extension of calcinosis, but, in some cases, colchicine, intravenous Ig and rituximab induced a subjective improvement in symptoms related to calcinosis.

Disclosure of Interest None declared

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