BackgroundScleroderma is a systemic autoimmune disease characterized mainly by skin manifestations and involvement of various visceral organs, especially the lungs. Lung involvement is the leading cause of mortality in patients with scleroderma. There are data to suggest that cyclophosphamide (CYC) and mycophenolate mofetil (MMF) are effective in the management of scleroderma interstitial lung disease (SSc-ILD) but no head to head comparative data are available to date.MethodsFor the last 3 years, patients with SSc-ILD have been treated at our centre by protocol-based administration of intravenous CYC and MMF. Results of lung function tests (spirometry) were recorded at baseline, 3 months and 6 months in every patient. The clinical records of patients with systemic sclerosis and significant ILD, who were not previously exposed to any immunosuppressant and were treated with MMF OR CYC, were reviewed. The efficacy of treatment was assessed by the change in forced vital capacity on spirometry.ResultsOf the total 57 patients included in the analysis, 34 were treated with MMF and 23 were treated with CYC. Mean duration of illness was 4.19 ± 2.82 years in the MMF and 6.04 ± 5.96 years in the CYC group. After 6 months of therapy, FVC increased by 10.84 ± 13.81 % in the CYC group and by 6.07 ± 11.92 % in the MMF group. This improvement from baseline was statistically significant in both groups (P < 0.01). The improvement was comparable with no statistically significant differences between groups (P = 0.373). There were no major adverse events reported in either arm.ConclusionBoth MMF and CYC were equally effective in stabilizing lung function in patients with scleroderma and ILD.
BackgroundPalindromic rheumatism (PR) although often considered as a benign disease can be severe and resistant to DMARDs in some patients. In these patients it can result in almost daily attacks, migrating from joint to joint resulting in poor quality of life. Rituximab has been proven to be effective in treatment of seropositive RA.ObjectivesTo determine the efficacy and safety of Rituximab in patients with seropositive PR who had an inadequate response to CsDMARDsMethodsPR was diagnosed based on criteria proposed by Hannonen P et al. Seropositive (ACPA±RF positivity) PR patients who had active disease despite being treated with two Cs DMARDs for >3 months, were treated with Rituximab. Active disease was defined as >4 attacks per month requiring intake of NSAIDS. All the patients were started on 500mg of rituximab after baseline work up. If complete control of palindromic attacks was not achieved and B cells were detectable in the peripheral blood by flow cytometry another 500 mg infusion was given after 2 weeks. Patients were continued on maximum tolerable dose of DMARDS. Patients were given repeat infusion of Rituximab once the patient developed clinical relapses as evidenced by recurrence of palindromic attacks.ResultsTwenty three patients with a mean age of 44.60±13.51 yrs and mean disease duration of 5.47±3.25 yrs were included. All patients were positive for ACPA while 17 patients were positive for RF. These patients were on a background of minimum of 2 DMARDs. Despite the maximum tolerable dose of DMARDs they had mean attack rate of 5.30±2.38 attacks per month. During a mean follow up of 14.17±8.62 months seven patients required two infusions and three patients required three infusions. Of the 33 infusions 500 mg was effective in controlling the attacks majority (88%) of the times. Seven patients required another 500 mg infusion after 2 weeks as initial 500 mg dose failed to achieve complete control of disease and B cell were not depleted in the peripheral blood. At one month follow up all patients achieved complete control of disease. Remission lasted for 10.33±5.75 months. When symptoms recurred patients were treated with rituximab again and all regained complete control of the symptoms. None of the patients evolved into RA during the study period. No serious adverse events were observed. Five patients experienced minor allergic reactions during infusion which were managed according to the standard protocol.ConclusionsThis case series indicates in patients of PR resistant to Cs DMARDs rituximab not only achieves disease control but also prevents progression to RA. To best of our knowledge this is the first report regarding efficacy of rituximab in PR. Although it needs to be proved in a larger blinded RCT this early data indicates that Rituximab may be a therapeutic option to prevent development of RA in seropositive patients.References Hannonen P, Möttönen T, Oka M. Palindromic rheumatism. A clinical surveyof sixty patients. Scand J Rheumatol. 1987;16(6):413–420.Sanmarti R, Cañete JD, Salvador G. Palin...
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