Morfeia e Terapia Hormonal: Uma Associação Possível

Morphea is a rare inflammatory disorder with an unknown etiology. We report the case of a 35-years-old woman presenting with an 18-month history of skin thickening on the extremities and trunk, and inflammatory arthralgia. Complaints started 2-weeks after a fertility treatment. The physical exam and workup confirmed the diagnosis of morphea, and the patient started treatment with deflazacort and methotrexate, with significant improvement. Six years later, the patient was submitted to another fertility treatment with exacerbation of the disease. The temporal association between the fertility treatments and the onset and further worsening of morphea suggest an influence of sex hormones on its pathophysiology.A morfeia é uma doença inflamatória, rara, de etiologia desconhecida. Apresentamos o caso de uma mulher de 35 anos com áreas de espessamento cutâneo no tronco e membros, e artralgias inflamatórias, com 18 meses de evolução. Os sintomas começaram 2 semanas após um tratamento de fertilidade. O exame físico e exames complementares confirmaram o diagnóstico de morfeia, pelo que iniciou deflazacorte e metotrexato com melhoria significativa dos sintomas. Seis anos depois, a doente realizou um novo tratamento de fertilidade com agravamento da doença. A associação temporal entre os tratamentos de fertilidade e o início e agravamento da morfeia sugerem uma influência das hormonas sexuais na sua fisiopatologia

More Than Just a Case of Polymyalgia Rheumatica

Malignant neoplasms are associated with a wide range of paraneoplastic rheumatological syndromes. These can be defined as remote effects of cancer, which are not caused by the tumor and its metastasis. The authors describe the case of an 82-year-old man, who presented with a one-month history of pain, stiffness, and functional limitation of the scapular and pelvic girdles. Blood tests showed raised levels of inflammatory markers. He was diagnosed with Polymyalgia Rheumatica, but the lack of response to corticosteroids led to the suspicion of an alternative diagnosis, like, for example, an occult neoplasm. Although patient evaluation was initially normal, five months later he developed macroscopic haematuria and was diagnosed with bladder and prostate carcinomas. After surgical treatment, the patient fully recovered from his rheumatological syndrome

Influence of the different “patient global assessment” formulations on disease activity score by different indices in rheumatoid arthritis

© 2018, International League of Associations for Rheumatology (ILAR). Patient global assessment (PGA) is included in almost all rheumatoid arthritis (RA) composite disease activity indices and definitions of remission. However, different PGA formulations exist and are used interchangeably in research and clinical practice. We investigated how five different PGA formulations used in four disease indices affect the remission rates. This was an ancillary analysis of data from a cross-sectional study in patients with RA. The data comprised the following: 28-joint counts, C-reactive protein, and five PGA formulations. Remission rate variation was assessed using five PGA formulations in each index (ACR/EULAR Boolean, CDAI, SDAI, and DAS28-CRP). PGA agreement was assessed by the following: Pearson’s correlation; Bland-Altman plots; paired samples t test; and establishing the proportion of patients who scored (i) all formulations within an interval of 20mm and (ii) each formulation ≤ 10mm. This analysis included 191 patients. PGA formulations presented good correlations (≥ 0.65), but Bland-Altman plots showed clinically significant differences, which were statistically confirmed by comparison of means. Just over a half (51.8%) of patients scored all PGA formulations within a 20-mm interval. The proportion of those scoring ≤ 10mm varied from 11.5 to 16.2%. When different formulations of PGA were used in each index, remission differences of up to 4.7, 4.7, 6.3, and 5.2% were observed. When formulations were used in their respective indices, as validated, the remission rates were similar (13.1, 13.6, 14.1, and 18.3%). Using PGA formulations interchangeably may have implications in the assessment of disease activity and in the attainment of remission, and this can impact upon management decisions

Management of infections in rheumatic patients receiving biological therapies. The Portuguese Society of Rheumatology recommendations.

Introduction: Infections are a major cause of morbidity and mortality in systemic inflammatory rheumatic diseases and the management of infectious complications in patients under biological therapies deserves particular attention. Objective: Develop evidence-based recommendations for the management of infections in rheumatic patients receiving biological therapies. Methods: A search in PubMed (until 10 November 2014) and EMBASE (until 20 December 2014) databases was performed. Patients with systemic inflammatory rheumatic diseases treated with approved biologics in whom infections occurred were included. Search results were submitted to title and abstract selection, followed by detailed review of suitable studies. Information regarding presentation of the infectious complication, its diagnosis, treatment, and outcome, as well as maintenance or discontinuation of the biological agent was extracted and subsequently pooled according to the type of infection considered. Results of literature review were presented and critically reviewed in a dedicated meeting by a multidisciplinary panel. Recommendations were then formulated using the Delphi method. Finally, the level of agreement among rheumatologists was voted using an online survey. Results: Fifteen recommendations were issued. Nine general recommendations concerned the assessment of infectious risk before and while on biologics, the procedures in case of suspected infection and the management of biologics during infectious complications. Six specific recommendations were developed for respiratory, urinary, gastrointestinal, skin, osteoarticular and disseminated infections. Conclusion: These fifteen recommendations are intended to help rheumatologists in the management of infections in patients on biological therapy. They integrate an extensive literature review, expert opinion and inputs from Portuguese rheumatologists