Systemic sclerosis with anti-RNA polymerase III positivity following silicone breast implant rupture: possible role of B-cell depletion and implant removal in the treatment.

Despite some case reports and small series of women with silicone breast implants (SBI) developing Systemic Sclerosis (SSc), no clear evidence of an association of SBI with SSc is available. However, SSc is characterized by clinical and immunological heterogeneity and autoantibodies are currently the best markers to stratify this heterogeneity of patients. Therefore, we have reviewed the literature for details of autoantibody characterization in reports of SSc associated with SBI. Moreover, the case of an anti-RNA polymerase III-positive SSc with rapid onset and progression, in which SBI rupture was found is described. This case may support a previous observation suggesting a possible role of SBI rupture as a trigger for anti-RNA polymerase III-positive SSc. This possible causal role may be reinforced by the observation that in our patient, despite immunosuppressive treatment, the disease progressed until SBI were removed, and reduction of anti-RNA polymerase III titer was obtained after rituximab treatment. This result may support data suggesting that B-cell depleting therapy may decrease specific autoantibody level in SSc patients, and that these changes are associated with disease improvement.

Health-related quality of life measured by the Short Form 36 (SF-36) in systemic sclerosis: correlations with indexes of disease activity and severity, disability, and depressive symptoms.

The aim of this study was to evaluate health-related quality of life (HR-QOL) in patients with systemic sclerosis (SSc), to compare it with that of patients with rheumatoid arthritis (RA), and to correlate it with other parameters. HR-QOL was evaluated by the Short Form 36 (SF-36), SSc disease activity and severity by preliminary indexes recently proposed, disability by the Health Assessment Questionnaire (HAQ), and depressive symptoms by the Beck Depression Inventory. HR-QOL perception was not statistically different in patients with SSc and RA, except that patients with diffuse cutaneous involvement had worse scores in the general health and mental health dimensions than patients with RA (p=0.03). Compared with RA, patients with SSc tended to perceive less bodily pain (p=0.06) and have less disability (p=0.04) but to report higher depressive symptom scores (p=0.05). SSc patients' HR-QOL was associated with some disease severity scales (general, kidney and, less significantly, heart), but it was poorly correlated with the other evaluated disease activity and severity indexes. A strong correlation with disability and with depressive symptoms was observed. In conclusion, patients with SSc perceived a reduced HR-QOL similar to that of patients with RA. SF-36 may provide useful information in their evaluation.

Dermatomyositis associated with lymphoproliferative disorder of NK cells and occult small cell lung carcinoma.

We report a case of dermatomyositis that was associated with a marked expansion of circulating NK cells (CD3-CD16+) with homogeneous phenotype (CD56- CD94- CD159a- CD244+) which satisfied the updated diagnostic criteria for the lymphoproliferative disorder of granular lymphocytes/NK cells. Immunosuppressive treatment induced remission of the dermatomyositis and decreased the number of circulating NK cells. However, a small cell lung carcinoma, that was occult at the time of the presentation of dermatomyositis, was diagnosed 10 months thereafter.

An individualized rehabilitation program in patients with systemic sclerosis may improve quality of life and hand mobility.

Few data are available to assess the efficacy of rehabilitative interventions in systemic sclerosis (SSc). We refer here the results of an individualized rehabilitation program in 16 patients with SSc. In particular, when possible, the number of patients who achieved a minimal clinically important difference (MCID) was determined. Results were evaluated taking advantage of the development of validated questionnaires and tests to assess quality of life (QOL) and disability in SSc. At the end of a period of 4 months of observation, 69% and 62% of patients reported an improvement of the physical and mental components of the SF-36 higher than the MCID (as established in other rheumatic conditions). Analogously, an improvement of the impact of respiratory disease on patients' QOL, as assessed by the Saint George's Respiratory Questionnaire, was perceived by 67% of them. These results might be explained by better exercise tolerance, which was suggested by the significant reduction of the heart rate and of a visual analogue scale for dyspnoea at the end of the 6-min walking test. Finally, a statistically significant improvement of hand mobility, as assessed by the hand mobility in scleroderma test was obtained. This study suggests that a significant proportion of patients with SSc experience an improvement in their perception of QOL, a better exercise tolerance, and a better hand mobility after a rehabilitation program consisting by a 2-week period of daily individual 30-min sessions as outpatient, followed by at-home exercise program.