Lymphocytic myocarditis in an overlap syndrome of systemic sclerosis and polymyositis

Clinical Rheumatology -     

Assessment of calcinosis in Portuguese patients with systemic sclerosis — a multicenter study

Clinical Rheumatology - The study aims to define the clinical and subclinical calcinosis prevalence, the sensitivity of radiographed site and clinical method for its diagnosis, and the phenotype of...     

Polyarticular lipoma arborescens—a clinical and aesthetical case

Lipoma arborescens is a benign tumor, but it may be a reactive process to other disorders, and its clinical, analytical, radiological and ultrasound presentation may be redundant to any synovial tumor. Despite the characteristic feature on magnetic resonance imaging (MRI), the correct differential diagnosis in atypical presentation, and the need for timely removal of the lesion to prevent joint damage, forces, ultimately, to invasive procedures. The clinical case reported here, fourth described in English language publications on the polyarticular form, also presented other specificities related to one of the swellings, in the knee. Because of its atypical location in the popliteal fossa, recurrent episodes of joint effusion, personal history of knee trauma, pulmonary tuberculosis, and family history of rheumatoid arthritis required particular attention. This process was hampered by the refusal of knee (and ankle) surgery by the patient. He accepted surgical removal of the swellings of the wrists, for aesthetical reasons, with pathologic confirmation of the diagnosis, and clinical success in that location. MRI of the knee showed the typical image of lipoma arborescens, but also other changes that compromise the prognosis.     

Food preferences of middle aged and elderly subjects in a Brazilian city

Objectives

The objectives of this study were to assess data on the consumption frequency of some food items and the places they are purchased, especially by adults and elderly subjects, and finally to determine their food preference using cluster and correspondence analyses as a diagnosis and prevention factor.

Design

Cross-sectional study.

Setting

Town of Serranópolis do Iguaçú, State of Paraná, Brazil, city with 4,854 inhabitants of which 22% are over 50 years old.

Participants

Two hundred and ninety five randomly selected subjects (96 men and 199 women), between 51 and 91 years old.

Measurements

A socio-demographic and food frequency questionnaire was applied via home visits including questions on socioeconomic, anthopometric and health characteristics concerning the consumption frequency of 45 food items and the places they were purchased, using a five point category scale to obtain consumption data as well. The body mass index (BMI) was calculated using the Quetelet Index and compared with the World Health Organization Report Consultation on obesity. The cluster and correspondence statistical analysis were used to determine their food preference.

Results

From the correspondence mapping (which explained 89.1 % of the variance), it was possible to determine aspects regarding the non-consumption of instant soups, frozen vegetables, cookies, crystallized fruits, canned fruit, distilled drinks and beer.

Conclusions

The dimensional representation structure demonstrated that the population of Serranópolis, who were 62 % overweight, preferred high fat content products, suggesting a reevaluation of their eating habits in the expectation of preventing non transmissible chronic diseases. The value given to farm production in Serranópolis do Iguaçú was also observed.     

Investigation of oxidative stress parameters in treated phenylketonuric patients

Phenylketonuria (PKU) is the most frequent disturbance of amino acid metabolism being caused by severe deficiency of phenylalanine hydroxylase activity. Untreated PKU patients present severe mental retardation whose pathophysiology is not completely estabilished. Despite the low-Phe diet, a considerable number of phenylketonuric patients present a mild to moderate psychomotor delay and decreased cognitive functions. In the present study we evaluated various parameters of oxidative stress namely thiobarbituric acid-reactive species (TBA-RS), total antioxidant reactivity (TAR) and activities of the antioxidant enzymes catalase (CAT), superoxide dismutase (SOD), and glutathione peroxidase (GSH-Px) in two groups of treated PKU patients, one with well controlled and the other with high Phe blood levels in order to investigate whether blood Phe concentrations could be correlated with the extend of oxidative stress. We initially verified a marked increase of TBA-RS, and a decrease of TAR in plasma, as well as a reduction of erythrocyte GSH-Px activity which were similar in both groups of PKU patients, when compared to controls of similar ages. In contrast, CAT and SOD activities were not altered in PKU patients. These results show that oxidative stress occurs in PKU patients and that this pathogenic process is probably not directly correlated to Phe blood levels.     

Hansen's disease mimicking a systemic vasculitis

Hansen's disease, caused by Mycobacterium leprae, classically presents with cutaneous and neurological manifestations. Rheumatologic manifestations present in 1 to 5% of the patients, and include arthritis, arthralgias, Charcot arthropathy, erythema nodosum and vasculitis. We report a case of a 86 year old woman with polyarthritis, subcutaneous nodules and leg ulcers whose differential diagnosis included primary vasculitis and diffuse connective tissue diseases and ended to be leprosy in a non endemic country.     

Clinical features and long-term outcomes of systemic lupus erythematosus: comparative data of childhood,adult and late-onset disease in a national register

Systemic lupus erythematosus (SLE) affects predominantly women at reproductive age but may present at any age. Age at disease onset has a modulating effect on presentation and course of disease, but controversies persist regarding its impact on long-term outcome. Our aims were to characterize clinical features, co-morbidities and cumulative damage in childhood-onset, adult-onset and late-onset SLE. Patients with childhood-onset SLE fulfilling ACR 1997 criteria were identified in a nationwide register-Reuma.pt/SLE (N = 89) and compared with adult-onset and late-onset counterparts matched 1:1:1 for disease duration. 267 SLE patients with mean disease duration of 11.9 ± 9.3 years were analyzed. Skin (62 %), kidney (58 %), neurological (11 %) and hematologic involvement (76 %) were significantly more common in childhood-onset SLE and disease activity was higher in this subset than in adult- and late-onset disease (SLEDAI-2K 3.4 ± 3.8 vs. 2.2 ± 2.7 vs. 1.6 ± 2.8, respectively; p = 0.004). Also, more childhood-onset patients received cyclophosphamide (10 %) and mycophenolate mofetil (34 %). A greater proportion of women (96 %), prevalence of arthritis (89 %) and anti-SSA antibodies (34 %) were noted in the adult-onset group. There was a significant delay in the diagnosis of SLE in older ages. Co-morbidities such as hypertension, diabetes and thyroid disease were significantly more frequent in late-onset SLE, as well as the presence of irreversible damage evaluated by the SLICC/ACR damage index (20 vs. 26 vs. 40 %; p < 0.001). Greater organ involvement as well as the frequent need for immunosuppressants supports the concept of childhood-onset being a more severe disease. In contrast, disease onset is more indolent but co-morbidity burden and irreversible damage are greater in late-onset SLE, which may have implications for patients’ management.