Management of calcinosis in rheumatic diseases.
| Treatment | Dosage | Study Design | Partial Response, n (%) | Complete Response, n (%) | First Author, Year | No. of Patients (Diseases) | Outcomes |
|---|---|---|---|---|---|---|---|
| Warfarin | 1 mg/d | RCT | 0 (0) | 0 (0) | Berger, 198749,a | 8 (4 placebo; 4 DM, SSc) | No regression of calcinosis |
| 1 mg/d | R | 0 (0) | 0 (0) | Lassoued, 198850 | 6 (DM, SSc) | Worsening of calcinosis, 1 stable | |
| 1 mg/d | R | 0 (0) | 2 (66) | Cukierman, 200451 | 3 (SSc) | 2 complete regressions of calcinosis | |
| NA | R | 1 (25) | 0 (0) | Balin, 201244 | 4 (SSc, DM) | 1 partial response in calcinosis | |
| NA | R | 0 (0) | 0 (0) | Fredi, 201545 | 2 (DM) | No response in calcinotic lesion | |
| Diltiazem | 60 mg tid | R | 3 (25) | 0 (0) | Vayssairat, 19988 | 12 (SSc) | 3 radiographic improvement |
| < 480 mg/d | R | 9 (53) | 0 (0) | Balin, 201244 | 17 (SSc, DM) | 10 cutaneous lesion improvement | |
| NA | R | 0 (0) | 0 (0) | Fredi, 201545 | 12 (DM) | No response in calcinotic lesion | |
| 240-480 mg/d | CS | 2 (50) | 2 (50) | Palmieri, 199542 | 4 (CTD) | Regression of calcific lesion | |
| 120 mg bid | CR | NA | 1 (100) | Dolan, 199543 | 1 (SSc) | Remission of calcinosis | |
| 240 mg/d | CR | 1 (100) | NA | Farah, 199041 | 1 (SSc) | Regression of calcinotic lesion | |
| Rituximab | 0.575-1 g/m2 wk 0/1 | RCT | NA | 1 (14) | Aggarwal, 201756,b | 76 (DM), 48 (JDM) | No improvement in calcinosis |
| 500 mg/m2 wk 0/2 | P | 0 (0) | 3 (100) | Moazedi-Fuerst, 201552 | 3 (SSc) | Regression of calcinotic lesion | |
| 500mg/m2 wk 0/2 | P | 4 (36) | NA | Narváez, 201453 | 9 (SSc) | Reduction in calcinotic lesion | |
| 375 mg/m2/wk × 4 | P | 3 (50) | NA | Giuggioli, 201554 | 10 (SSc) | Improvement in calcinosis in 3/6 patients | |
| 2 × 500 mg/m2 | P | 0 (0) | 0 (0) | Bader-Meunier, 201155 | 6 (JDM) | No calcinosis improvement in 6 patients | |
| 375 mg/m2 × 4 | CR | NA | 1 (100) | Daoussis, 201257 | 1 (SSc) | Calcinosis significantly improved and pain resolved | |
| Bisphosphonate | NA | R | 1 (20) | 0 (0) | Balin, 201244 | 5 (DM, SSc) | 1 partial response, 3 had no response |
| IV 1 mg/kg/d | R | 2 (66) | 1 (33) | Marco Puche, 201062 | 3 (JDM) | Reduction and remission of calcinosis | |
| IV 1 mg/kg/d | R | 2 (33) | 2 (33) | Tayfur, 201561 | 6 ( JDM) | Resolution of calcinosis in 4/6 patients | |
| 10 mg/kg/d | CR | 1 (100) | NA | Rabens, 197564 | 1 (SSc) | Partial regression of calcinosis | |
| 10 mg/d | CR | 1 (100) | NA | Masza Mukamel, 200163 | 2 (JDM) | Complete resolution of calcified lesions | |
| Initial dose 10 mg/kg/d, then 20 mg/kg/d | CR | 0 (0) | 0 (0) | Metzger, 197465 | 6 (SSc, DM) | Progression of calcinosis | |
| Surgical excision | NA | R | 3 (27) | 8 (73) | Balin, 201244 | 11 (DM, SSc) | Complete response in 8/11 patients |
| Extracorporeal shock wave lithotripsy | NA | P | 4 (100) | 0 (0) | Blumhardt, 201691 | 4 (SSc) | Reduction of calcinosis |
| NA | P | 1 (33) | 0 (0) | Sultan-Bichat, 201290 | 4 (venous insufficiency), 1 (DM), 3 (SSc) | Reduction of calcinotic lesion | |
| Carbon dioxide laser | P | P | 5 (83) | NA | Bottomley, 199689 | 6 (SSc) | Pain reduction |
| Iontophoresis of acetic acid | Iontophoresis with 2-5% acetic acid at 10 microA for 20 minsc | P | 0 (0) | 0 (0) | Shetty, 200577 | 3 (SSc) | Reduction in the intensity of calcinosis in imaging, but no clinical benefits |
| Surgical excision (microdrilling) | NA | P | 12 (80) | NA | Fahmy, 199888 | 15 (SSc) | Improvement of calcinosis in 12/15 digits |
↵aIn Berger et al,49 4 patients received placebo, 3 received low-dose warfarin, and 1 was excluded for noncomplianace. The outcome was extent of calcinosis based on clinical and radiographic examination.
↵bIn Aggarwal et al,56 the primary endpoint was the cutaneous lesions (skin rashes) and not the calcinosis.
↵cIontophoresis was followed by ultrasound at 1.5 W/cm2 for 8 min occurring 9 times over a 3-week period. Bid: twice daily; CR: case report; CS: case series; CTD: connective tissue diseases; DM: dermatomyositis; IV: intravenous; JDM: juvenile dermatomyositis; NA: not applicable; P: prospective case series; PCT: placebo-controlled trial; R: retrospective case series; RCT: randomized controlled trial; SSc: systemic sclerosis; tid: three times daily.