Systemic sclerosis
Treatment of Systemic Sclerosis-Associated Calcinosis: A Case Report of Rituximab-Induced Regression of CREST-Related Calcinosis and Review of the Literature

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Objectives

Calcinosis is frequently encountered in patients with systemic sclerosis (SSc) and may be associated with significant morbidity. No treatment has shown so far an unequivocal beneficial effect.

Methods

We performed an extensive internet search (MEDLINE) using the keywords calcinosis, calcification, scleroderma, systemic sclerosis, and treatment.

Results

Our patient had extensive Calcinosis, Raynaud, Esophagitis, Sclerodactyly, telangiectasia (CREST)-related calcinosis, frequently ulcerating and painful. Following 2 rituximab courses (consisting of 4 weekly infusions, 375 mg/m2 each), calcinosis significantly improved and pain disappeared. Pharmacologic agents used in the treatment of SSc-associated calcinosis include diltiazem, minocycline, warfarin, biphosphonates, and intravenous immunoglobulin. Other therapeutic approaches include surgical excision, laser vaporization, and extracorporeal shock wave lithotripsy.

Conclusions

Evidence for all existing therapies is weak and therefore larger scale controlled studies are needed. Rituximab appears as a promising treatment especially in view of recent evidence that this therapy may be also effective in the underlying disease.

Section snippets

Methods

We performed an extensive Internet search (Medline) using the keywords calcinosis, calcification, scleroderma, systemic sclerosis, and treatment in the following combinations: systemic sclerosis (or scleroderma) and calcinosis (or calcification) and treatment. The limits used were (1) English language, (2) human subjects, and (3) published from 1981 and onward. The search identified 392 articles; following duplicate removal, 182 articles remained and were screened for eligibility. One hundred

Literature Review

In this section, we review all the available pharmacologic (summarized in Table 1) and nonpharmacologic treatment options for SSc-associated calcinosis.

Discussion

Treatment of SSc-associated calcinosis has been so far disappointing. Even though several modalities have been tried, none has definitely shown a beneficial effect. We present herein the first case of RTX administration in limited SSc; all patients recruited so far in the studies assessing the efficacy of RTX in SSc belonged to the diffuse form of the disease. The rationale for the use of RTX in SSc is based on experimental evidence from animal models, indicating that B cells may be actively

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    These authors contributed equally to this article.

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