From the Guidelines
Local calcification in scleroderma should be managed with a combination of topical and oral treatments, prioritizing patient safety and quality of life, as there is limited evidence to guide clinicians on the treatment of SSc-related calcinosis 1.
Treatment Approaches
- For mild cases, topical treatments like sodium thiosulfate 10-25% applied once or twice daily for several months may help dissolve calcium deposits.
- For more significant calcifications, oral medications including diltiazem (30-60mg three times daily), minocycline (50-100mg twice daily), or warfarin (dose adjusted to maintain INR 2-3) may be beneficial over 3-6 months.
- Colchicine (0.6mg once or twice daily) can also reduce inflammation around calcifications.
- For painful or infected lesions, surgical excision may be necessary.
- Extracorporeal shock wave therapy can be considered for recalcitrant cases.
Important Considerations
- Patients should also protect affected areas from trauma, maintain good skin hygiene, and promptly treat any skin infections.
- Regular follow-up is important as calcifications may recur, requiring ongoing or repeated treatment.
- The effectiveness of these treatments varies among individuals, and a combination approach is often needed for optimal results.
- According to the 2023 EULAR recommendations for the treatment of systemic sclerosis, novel interventions for the management of calcinosis are needed, and research should prioritize this area 1.
- The use of immunosuppressive therapy, such as mycophenolate mofetil, rituximab, and tocilizumab, may be beneficial for skin fibrosis and interstitial lung disease, but their role in calcinosis is still uncertain 1.
From the Research
Local Calcification in Sclerodermia
- Localized scleroderma (LS) is characterized by excessive collagen deposition leading to thickening of the dermis, subcutaneous tissue or both 2.
- Calcinosis, or dystrophic soft-tissue calcification, occurs in damaged or devitalized tissues in the presence of normal calcium/phosphorus metabolism, and is often noted in the subcutaneous tissues of connective tissues diseases, including scleroderma 3.
- Calcinosis cutis, defined as sub-epidermal deposition of calcium salts, is a major clinical problem in patients with systemic sclerosis (SSc), affecting 20-40% of patients 4.
Pathogenesis and Treatment
- The pathogenesis of SSc-related calcinosis remains unknown, and currently there is no effective disease-modifying pharmacotherapy 4.
- A number of recognized factors associated with calcinosis have been identified, including disease duration, digital ischaemia and acro-osteolysis 4.
- Treatment of calcinosis in scleroderma patients is aimed toward relief of symptoms, and may include surgical treatment, warfarin, colchicine, probenecid, bisphosphonates, diltiazem, minocycline, aluminum hydroxide, salicylate, and carbon dioxide laser therapies 3, 5.
Characterization of Calcinosis
- X-ray diffraction analysis of spontaneously draining calcinosis in scleroderma patients confirmed that calcium hydroxyapatite (HA) was the only crystalline material, but HA was the minor component and most samples contained more than 50% organic material 6.
- The correct diagnosis of calcinosis is crucial for successful treatment, and patient approach and treatment vary greatly depending on the cause of soft-tissue calcifications 5.