Table 1.
Existing Pharmacological Therapies for Calcinosis in Dermatomyositis
| Name of drug | Dose | Mechanism of action | Side effects | Level of evidence/source |
|---|---|---|---|---|
| Diltiazem (oral) | 330-360mg/day in adult DM 5-6mg/kg/day in juvenile DM |
Reduces calcium efflux from dystrophic muscle | Malaise, Nausea, headache, lower extremity edema, and lowering the blood pressure. | Bertorini TE et al(47) Vinen CS et al(48) Abdallah Lotf et all (49) S.J. Balin et al(50) |
| Amlodipine (oral) | 10mg a day | Peripheral vasodilation | Same as above | S.J. Balin et al(50) |
| Sodium thiosulfate (topical) | 25% sodium thiosulfate compounded in zinc oxide ointment-applied 2 to 3 times daily to affected areas (base can be substituted with cold cream or petrolatum) | Chelates calcium, enhances solubility and clearance of calcinosis | Transient burning sensation after application | Topham C et al(57) Eleryan MG et al (58) Del Barrio-Díaz P et al (59) Ma JE et al(60) Nowaczyk J et al(61) |
| Sodium thiosulfate (intralesional) | Volume-0.1ml/cm^2 of affected area Concentration-40 mg/ml | Infection, Local pain or transient burning sensation following injection | Goossens J et al(62) Baumgartner-Nielsen J et al(63) López-Sundh AE et al(64) Winter AR et al(69) |
|
| Sodium thiosulfate (intravenous) | 12.5g-25g sodium thiosulfate three times a week | Fatigue, nausea, vomiting, metabolic acidosis not requiring treatment | Badawi AH et al(68) Mageau A et al(69) Song P et al(70) |
|
| Bisphosphonates Pamidronate (intravenous) | 1mg/kg/day for 3 days and given every 3 months | Inhibit macrophage activity, decrease bone turnover, prevent formation of calcium phosphate crystals | flu-like symptoms in first 24-72 hrs after infusion | Tayfur AC et al(75) Marco Puche A et al(76) Slimani S et al(77) I. Saini et al(78) |
| Alendronate (oral) | 10mg/day | Esophagitis | M.L. Bianchi et al(80) | |
| Probenecid (oral) | 250mg/day 1.5g/day 1g/day |
Increase phosphate excretion- decrease serum calcium phosphate product | flushing, headache, dizziness, anorexia | Skuterud E et al(89) Eddy MC et al(90) Nakamura H et al(91) |
| TNF-alpha inhibitors-Infliximab (intravenous) | 3mg/kg at 0,2,6 week and then every 8 weeks | Suppression of cytokine release | Infection of calcinotic lesion, allergic reaction | Riley P et al(92) Campanilho-Marques R et al(93) Tosounidou S et al(94) |
| JAK-inhibitor Tofacitinib (oral) Baricitinib(oral) |
5mg twice a day or 11mg extended release od 6mg bid |
Decreases ROS-mediated mitochondrial calcium accumulation | Hypercalcemia, weight gain, vascular thrombosis, malignancy and serious infections | Wendel S et al(100) Betül Sözeri et al(101) Papadopoulou C et al(102) Sabbagh S et al(103) |
| Rituximab (intravenous) | 375-500 mg/m^2 body surface area, 2-4 infusions | Suppress immunity via anti-CD20 action | Infections | Bader-Meunier B et al(96) |
| IVIG | 2g/kg/month given over 3-4 days | Competes with C5-C9 complex deposition in tissues | Headache, nausea, thrombosis | Aggarwal R et al(107) Touimy M et al(108) Peñate Y et al(109) Shahani L et al(110) Galimberti F et al(111) |
| Warfarin (oral) | 1mg/day | Reduces gamma carboxylation | Monitor INR if bleeding happens | Cukierman T et al(123) |
| Minocycline (oral) | 50-100mg/day | Anti-inflammatory and anti-microbial | Dizziness, fatigue | Robertson LP et al(103) S.J. Balin et al(50) Carmen Fonseca et al(104) |
| Treprostinil (oral) | 0.125mg three times a day and can be increased gradually as tolerated | Prostaglandin analogue/vasodilator |
Headache and gastrointestinal disturbance like GERD | Chung, Melody et al(118) |