Points to consider for designing trials in systemic sclerosis patients with arthritic involvement.

Although musculoskeletal involvement is quite common in SSc (arthritic in particular), there have been few trials and even fewer controlled trials of therapeutic agents in arthritis in SSc. In addition, there have been only three outcome measures that have been validated for use in trials of SSc arthritis: the HAQ Disability Index, the Cochin Hand Function Scale and the Hand Mobility in SSc scale. The purpose of this article is to present evidence-based points to consider for the design of trials in SSc patients with musculoskeletal involvement (joints in particular). In addition, we make an argument for including outcome variables that can be validated within a given trial for use in future trials.

Inter-rater reliability of modified hand mobility in scleroderma test.

AIM: Systemic sclerosis (SSc) is a chronic autoimmune disease of unknown etiology characterized by excessive collagen production, endothelial cell injury, microvascular obliteration, cutaneous fibrosis and progressive visceral disease. The hands are frequently involved during the progression of the disease, with symmetrical skin thickening as a prominent feature. Modified hand mobility in scleroderma (mHAMIS) test is a measurement method to assess hand mobility in patients with SSc. Knowing the inter-rater reliability of the instrument is important in order for the results from different examiners to be accurately interpreted. The aim of this study was to test inter-rater reliability of the mHAMIS test. METHOD: Hand mobility for both hands was assessed in 25 female patients with SSc by 2 physiotherapists who have different years of experience. Patients who had flexion contracture in at least 1 finger and undergone hand surgery in the last year due to any injuries, were excluded from the study since hand mobility was prevented. Inter-rater reliability was determined using intra-class correlation coefficients (ICCs). RESULT: The ICCs were excellent between raters for dominant and non-dominant hands. The values were 0.92 and 0.93, respectively. CONCLUSION: The inter-rater reliability of the mHAMIS was found to be excellent. This research contributes to the literature by proving that the test can be used without causing bias in clinical trials.

The Modified Hand Mobility in Scleroderma Test and Skin Involvement - A Followup Study.

OBJECTIVE: To study the change in the modified Hand Mobility in Scleroderma (mHAMIS) test from early to advanced stages of systemic sclerosis (SSc), and the relationship between mHAMIS and skin involvement during followup. METHODS: This retrospective study includes 65 patients with baseline disease duration of ≤ 3 years who were assessed with the mHAMIS test at baseline and at 1 or 2 predefined followup points (3.1-5 yrs and 5.1-9 yrs after disease onset). Studied measures were the modified Rodnan skin score (mRSS), mRSS of the hand, serum cartilage oligomeric matrix protein, and digital vascular lesions. RESULTS: The mHAMIS and the mRSS hand changed synchronously during the first 5 years after disease onset (rs = 0.44, p = 0.001). In the group with high mHAMIS at baseline, both mHAMIS and mRSS hand improved significantly at the first followup (p < 0.05), and the improvement sustained during the followup in the mRSS hand. Patients with antitopoisomerase I and anti-RNA polymerase III antibodies had significantly higher mHAMIS at baseline (p = 0.003) and at the second followup (p = 0.030) compared to patients with anticentromere antibodies. Patients with digital vascular lesions at baseline had significantly higher mHAMIS during the followup (p < 0.05) compared to patients without. The mHAMIS improved significantly during the followup in patients with immunosuppressive treatment in early disease (p < 0.05), but not in patients without this treatment. CONCLUSION: The mHAMIS reflects disease activity in fibrosis in early stages of SSc. In later stages it can be regarded as a measure of damage arising from fibrotic and vascular involvement, making it suitable as an endpoint in followup examinations.

Development of a modified hand mobility in scleroderma (HAMIS) test and its potential as an outcome measure in systemic sclerosis.

OBJECTIVE: To modify the hand mobility in scleroderma (HAMIS) test by reducing the number of items and amount of equipment needed, and to evaluate the construct validity of this modified HAMIS (mHAMIS). METHODS: Our retrospective study is based on 266 patients previously examined using the original HAMIS test. Data were divided into 3 groups depending on disease duration after onset: (1) 0-3 years, (2) 3.1-5 years, and (3) 5.1-9 years. Disease variables included were skin involvement using the disease subset and the modified Rodnan skin score (mRSS), and digital lesions. Cronbach's alpha coefficient was calculated separately for limited (lcSSc) and diffuse systemic sclerosis (dcSSc) for the right and left hand, and for the groups with different disease duration. The construct validity of the mHAMIS was assessed by searching for a correlation with hand skin score. RESULTS: An mHAMIS test consisting of finger flexion, finger extension, finger abduction, and dorsal extension was developed. The internal consistency of this test was 0.78, 0.83, and 0.73 in the 3 groups with different disease duration. In the whole study group, mHAMIS showed a significant correlation with mRSS and hand skin score (rs=0.39 and 0.43, respectively), and was able to discriminate between lcSSc and dcSSc (p=0.001), and between patients with and without ulcers (p=0.015). CONCLUSION: The mHAMIS involves 4 easily measurable items and has the potential to be a relevant clinical measure of outcome in the evaluation of fibrotic skin involvement in SSc.