Verônica Silva Vilela

Rituximab for the therapy of systemic sclerosis: a series of 10 cases in a single center

Systemic sclerosis (SSc) is a chronic autoimmune disease with a high morbidity and mortality. Although cyclophosphamide is effective for severe and refractory cases, there is demand for new treatments. The biological treatment with B-cell depletion with rituximab (RTX) has demonstrated efficacy for this demand in open-label studies. OBJECTIVE This study was conducted with the aim to retrospectively evaluate all patients who used RTX for the treatment of SSc in our center. PATIENTS AND METHODS We retrospectively evaluated medical records of all patients with SSc who used RTX to treat this disease from January 2009 to January 2015. Systemic, cutaneous, and pulmonary involvement data and laboratory results before and six months after the first infusion of RTX were collected. RESULTS Ten patients received treatment during the study period and were included in this series. All patients had a diffuse form of the disease. Five patients suffered from an early (duration of disease shorter or equal to four years), rapidly progressive disease, and another five received RTX at late stages of the disease. In both groups of patients, stabilization of the pulmonary picture was observed, with a fall in the skin score in those patients with early forms of the disease. DISCUSSION Similar to findings in previous studies, RTX was effective in treating early and rapidly progressive forms of SSc. We also found that patients with long-term illness may benefit from the treatment.

Recomendações para o tratamento da síndrome de Sjögren

The recommendations proposed by the Sjögrens Syndrome Committee of the Brazilian Society of Rheumatology for the treatment of Sjögrens syndrome were based on a systematic review of literature in Medline (PubMed) and the Cochrane databases until October 2014 and on expert opinion in the absence of studies on the subject. 131 items classified according to Oxford & Grade were included. These recommendations were developed in order to guide the appropriate management and facilitate the access to treatment for those patients with an appropriate indication, considering the Brazilian socioeconomic context and pharmacological agents available in this country.

Rapidly progressive diffuse systemic sclerosis after local vitamins A, D and E complex injections: literature review and report of two cases

The term autoimmune/autoinflammatory syndrome induced by adjuvants (ASIA) or Shoenfeld’s syndrome refers to a wide group of immune-mediated diseases triggered by external agents. Several substances, such as vaccine adjuvants, squalene and silicone implants, are implied in the pathogenesis of ASIA syndrome. Treatment and prognosis of this complex condition are not completely known due to lack of good quality evidence. After a brief introductory literature review on ASIA, we report here two cases of patients that developed rapidly progressive systemic sclerosis clinical features after multiple intramuscular local injections of a substance recommended by a non-medical professional called ADE. ADE is an oily vitamin complex for veterinary use, and it was used in these cases for cosmetic muscular definition and enhancement purpose. To our knowledge, this is the first paper to describe the relation between injections of ADE and the development of ASIA with severe systemic sclerosis phenotype. Further investigation is needed to better understand the pathophysiology and to provide the basis for the treatment of this condition.

Computed tomography trachea volumetry in patients with scleroderma: Association with clinical and functional findings

Background In scleroderma, excessive collagen production can alter tracheal geometry, and computed tomography (CT) volumetry of this structure may aid in detecting possible abnormalities. The objectives of this study were to quantify the morphological abnormalities in the tracheas of patients with scleroderma and to correlate these findings with data on clinical and pulmonary function. Methods This was a cross-sectional study in which 28 adults with scleroderma and 27 controls matched by age, gender and body mass index underwent chest CT with posterior segmentation and skeletonization of the images. In addition, all participants underwent pulmonary function tests and clinical evaluation, including the modified Rodnan skin score (mRSS). Results Most patients (71.4%) had interstitial lung disease on CT. Compared to controls, patients with scleroderma showed higher values in the parameters measured by CT trachea volumetry, including area, eccentricity, major diameter, minor diameter, and tortuosity. The tracheal area and equivalent diameter were negatively correlated with the ratio between forced expiratory flow and forced inspiratory flow at 50% of forced vital capacity (FEF50%/FIF50%) (r = -0.44, p = 0.03 and r = -0.46, p = 0.02, respectively). The tracheal tortuosity was negatively correlated with peak expiratory flow (r = -0.51, p = 0.008). The mRSS showed a positive correlation with eccentricity (r = 0.62, p < 0.001) and tracheal tortuosity (r = 0.51, p = 0.007), while the presence of anti-topoisomerase I antibody (ATA) showed a positive correlation with tracheal tortuosity (r = 0.45, p = 0.03). Conclusions In a sample composed predominantly of scleroderma patients with associated interstitial lung disease, there were abnormalities in tracheal geometry, including greater eccentricity, diameter and tortuosity. In these patients, abnormalities in the geometry of the trachea were associated with functional markers of obstruction. In addition, tracheal tortuosity was correlated with cutaneous involvement and the presence of ATA.

Ventilation distribution and small airway function in patients with systemic sclerosis

BACKGROUND Despite the importance of traditional pulmonary function tests (PFTs) in managing systemic sclerosis (SSc), many patients with pulmonary disease diagnosed by computed tomography (CT) present with normal PFTs. OBJECTIVE To evaluate the efficacy of the nitrogen single-breath washout (N2SBW) test in diagnosing SSc and to correlate N2SBW parameters with the PFT indexes used in the follow-up of these patients, clinical data, and CT findings. METHODS Cross-sectional study in which 52 consecutive SSc patients were subjected to spirometry, body plethysmography, analysis of the diffusing capacity for carbon monoxide (DLCO), analysis of respiratory muscle strength, N2SBW testing, and CT analysis. RESULTS Twenty-eight patients had a forced vital capacity (FVC) that was <70% of the predicted value. In the N2SBW test, 44 patients had a phase III slope (Phase III slopeN2SBW) that was >120% of the predicted value, while 15 patients had a closing volume/vital capacity (CV/VC) that was >120% of the predicted value. A significant difference in Phase III slopeN2SBW was observed when the patients with predominant traction bronchiectasis and honeycombing were compared to the patients with other CT patterns (p<0.0001). The Phase III slopeN2SBW was correlated with FVC (rs=-0.845, p<0.0001) and DLCO (rs=-0.600, p<0.0001), and the CV/VC was correlated with FVC (rs=-0.460, p=0.0006) and residual volume/total lung capacity (rs=0.328, p=0.017). CONCLUSION Ventilation heterogeneity is a frequent finding in SSc patients that is associated with restrictive damage, changes in pulmonary diffusion, and CT patterns. In addition, approximately one-third of the patients presented with findings that were compatible with small airway disease.

AB0533 Recommendations of Brazilian Society of Rheumatology for the Treatment of SjÖGren's Syndrome

Objectives Recommendations of Brazilian Society of Rheumatology for the treatment of Sjögrens syndrome (SS) were developed to guide management of SS considering the Brazilian social and economic context. Methods It was based on specialists opinion and systematic review on MEDLINE (PubMed) and Cochrane database until October 2014, including 127 articles classified according Oxford & Grade. Results Forty-four recommendations were organized in 3 main topics: Part 1. General recommendations and patient education: The management of SS should be conducted by multidisciplinary team. Systemic treatment should be according disease severity measured by EULAR Sjögrens Syndrome Disease Activity (ESSDAI). Patients should avoid caffeine, tobacco, alcohol, toothpaste with abrasive, and mouthrinses with alcohol. Patient should be educated about preventive measures for oral health and hydration. Aerobic exercise improves fatigue and quality of life. Immunization to influenza and pneumococo are indicated. Serum levels of vitamin D should be evaluated and supplemented if it is necessary. Part 2. Symptomatic treatment of dryness: Topical treatment for dry mouth includes saliva substitutes, sugar-free candies and gums. Topical treatments for dry eye are lubricants (glucanes or carboximethylcelulose), cyclosporine 0.05%, and punctual occlusion. Topical glucocorticoids may be used for severe dry eye for short time avoiding complications. Pilocarpine and cevimeline should be used for dry mouth and for severe dry eye. N-acetylcysteine may be used for dryness symptoms, including patients showing intolerance for muscarinic agonists. Omega-3 supplementation may be used to dry eye. Part 3. Systemic treatment: Immunosupressant and/or biological therapy are not indicated to dryness treatment. Hydroxichloroquine, glucocorticoid, and immunossupressants (azathioprine, mycophenolate mofetil, cyclophosphamide, cyclosporine) should be indicated according severity of systemic involvement. Rituximab is indicated to treat systemic manifestations without improvement with immunosuppressive therapy. Abatacept and belimumab may be considered in patients not responding to rituximab and with high level of disease activity. Acknowledgements Sociedade Brasileira de Reumatologia (SBR) Disclosure of Interest None declared