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.

Patients' satisfaction with the rheumatology day care unit.

BACKGROUND: Patients receiving biological therapies are regularly evaluated and monitored at rheumatology day care units (RDCU). Despite patients' satisfaction with the delivered care and the relationship between the patient and the multidisciplinary team being acknowledged as important aspects to ensure adherence to therapy, factors associated with them have not been investigated so far. OBJECTIVES: To evaluate patients' satisfaction with the functioning of the RDCU and to identify the factors associated with the level of satisfaction. METHODS: An anonymous questionnaire was administered to all patients with rheumatoid arthritis (RA) or spondyloarthritis treated with biological drugs and followed at the RDCU at Hospital Garcia de Orta, Almada, Portugal. Satisfaction was measured using a visual analogue scale (0-100, 0 meaning completely unsatisfied, 100 meaning completely satisfied). Further information was collected on socio-demographic variables, physical conditions of the RDCU, waiting time, satisfaction with the role of medical, nursing and administrative staff (satisfaction level with their friendliness, question answering, care delivery, privacy during consultation, clarity in the information given, which was then transformed into a composite score, 0-20). Factors associated with satisfaction were studied by univariable followed by multiple linear regression to adjust for potential confounders. RESULTS: In total, 150 patients were included in the study (mean age 50.6 ± 13.7 years, 64% female, 62% RA, mean disease duration 10.6 ± 6.1 years). The majority of patients attended the RDCU for more than three years and 57% received subcutaneous therapy. The mean level of satisfaction with the RDCU was 81.9 ± 17.9. Multivariable analysis showed that intravenous therapy (ß 6.13, 95% confidence interval - CI 0.71-11.55), physician score (ß 2.28, 95%CI 1.20-3.35) and increasing levels of satisfaction with the room temperature (ß 5.64, 95%CI 3.06-8.21) and waiting time (ß 25.53, 95%CI 8.17-42.89, for a very good vs non-acceptable waiting time) were positively associated with the level of satisfaction, while the nursing score was inversely associated. CONCLUSIONS: Patients were overall very satisfied with the functioning of the RDCU. Waiting time, satisfaction with the physician role, room temperature and intravenous therapy were the main factors positively associated with the level of satisfaction.

Assessment of salivary gland function in Sjögren's syndrome: the role of salivary gland scintigraphy.

Salivary gland scintigraphy (SGS) is a non invasive method of salivary gland function assessment. This technique is easy to perform, reproducible and well tolerated by patients. Additionally, an abnormal salivary gland scintigraphy result is accepted by the American-European consensus group as a criterion for the diagnosis of Sjögren's syndrome. Scintigraphic evaluation of salivary gland function also plays an important role in therapeutic decision and patient follow-up. Schall's categorical classification is usually considered the standard method for salivary scintigraphy interpretation, though subjective and with limited capacity to discriminate borderline results. In order to improve the diagnostic accuracy of SGS, there has been an increasing interest in the quantification of glandular function. However, the debate on the most reliable and suitable parameters for the diagnosis of SS persists.

[Salivary gland scintigraphy in the evaluation of patients with sicca complaints]. / Cintigrafia das glândulas salivares major na avaliação de doentes com queixas secas.

UNLABELLED: Salivary gland scintigraphy is a non invasive method widely accepted as an objective assessment of salivary gland function and a diagnostic criterion of primary Sjögren's Syndrome. In this study we evaluated the performance of qualitative and semi--qualitative salivary gland scintigraphy in primary Sjögren's Syndrome. Additionally we aimed to identify the scintigraphic patterns of salivary gland involvement in this condition. PATIENTS AND METHODS: Observational study of fifty--six women with suspected primary Sjögren's Syndrome (pSS). Investigation included clinical history and observation, ophtalmologic examination, autoantibodies (SSA and SSB) determination, minor salivary gland biopsy and salivary gland scintigraphy interpreted according to Schall classification, visual quality of radioactivity uptake, morphology of time-activity curves and semi-quantitative parameters of uptake and excretion. RESULTS: Twenty patients fulfilled diagnostic criteria for pSS. Submandibular glands showed more pronounced functional impairment. In pSS patients, all scintigraphic parameters were significantly lower on these glands: a) qualitative evaluation ' visual quality of uptake (p=0,003), morphology of time-activity curves (p=0,001) and Schall classification (p<0,001); b) semi-quantitative parameters ' maximum counts (p=0,005), maximum counts/second/MBq administered (p=0,01), index of maximum counts versus counts at first minute (p=0,002) and excretion index (p=0,006). CONCLUSIONS: Despite being qualitative and observer-dependent, Schall classification is valuable for the diagnosis of primary Sjögren's Syndrome. Nevertheless, semi-quantitative evaluation of salivary gland scintigraphy reveals significant differences in pSS patients and may have incremental value for the interpretation of salivary gland scintigraphy results.

[Diagnostic performance of anti-cyclic citrullinated antibodies and IgM rheumatoid factor in rheumatoid arthritis]. / Avaliaçao do comportamento dos anticorpos anti-citrulina e do factor reumatóide IgM na artrite reumatoide.

UNLABELLED: The presence of IgM class rheumatoid factor RF-M in the serum is one of the ACR criteria for the classification of rheumatoid arthritis RA . OBJECTIVES: To compare the sensitivity and specificity of two diagnostic markers for RA in Portuguese patients the 2nd generation anti-cyclic citrullinated peptide antibodies anti-CCP2 and the RF-M. METHODS: Serum samples from 56 patients with established RA 44 women and 12 men mean disease duration 9 5 -8 5 years were evaluated for the presence of anti-CCP2 and RF-M. Results were compared with 93 samples from a control group with identical demographic characteristics 43 from patients with spondylarthropaties and 50 from healthy blood donors . The determination of anti-CCP2 antibodies was performed by enzymatic fluroimunoassay and RF-M was measured by microElisa. RESULTS: Anti-CCP2 antibodies were positive in 40 patients with RA 71 4 at a mean antibody concentration of 350 5 UA ml range 11-1670 UA ml and negative in all controls. The RF-M was positive in 34 patients with RA 60 7 in 4 patients with spondylarthropaties 9 3 and in one blood donor 2 . The sensitivity specificity positive predictive value and negative predictive value for RF-M was 60 7 90 7 89 5 and 63 9 and for anti-CCP2 was 71 4 100 100 and 72 9 respectively. CONCLUSIONS: The anti-CCP2 assay showed an excellent specificity and a global performance superior to that of the RF-M test in this population with established RA.

[Flexibilization of infliximab dose interval in the treatment of ankylosing spondylitis]. / Flexibilização do Intervalo entre as Infusões de Infliximab no Tratamento dos Doentes com Espondilite Anquilosante.

OBJECTIVE: The aim of this study was to access the maintenance of therapeutic response in patients with ankylosing spondylitis (AS) receiving infliximab, after prolongation of the interval between infusions. MATERIAL AND METHODS: In AS patients with sustained therapeutic response to infliximab administered every six weeks, a prolongation of the interval between infusions was proposed. The therapeutic response was evaluated using Bath Ankylosing Spondylitis Disease Activity Index (BASDAI), Bath Ankylosing Spondylitis Functional Index (BASFI), pain, stiffness, patient's global evaluation and acute phase reactants (ESR and CPR). RESULTS: Twenty patients participated in the study. Sixteen were male, with a mean age of 40.3 +- 10.9 years and mean disease duration of 12 +- 9.4 years. The initial BASDAI was 59.5 +- 22.8 and initial BASFI 50.7 +- 27. The interval between infusions was lengthened to 8 +- 1 weeks (minimum 7; maximum 10) and this change occurred between 12 and 142 weeks (median 21) of treatment. The mean followup after changing the therapeutic regimen was 86 +- 45 weeks. Sixty-five percent (13/20) of the patients maintained an adequate response. CONCLUSIONS: Two thirds of AS patients with sustained therapeutic response to infliximab administrated every six weeks maintained an adequate response with prolongation of interval between infusions. Therefore the increase of the interval between infusions seems to be an adequate option in selected patients.

[Characterization of a Portuguese population with systemic lupus erytematosus]. / Caracterização de uma população Portuguesa de doentes com lupus eritematoso sistémico.

UNLABELLED: To characterize systemic lupus erythematosus (SLE) in Portuguese patients and to identify differences in diseases expression related to sex and ethnicity. PATIENTS AND METHODS: Retrospective cohort analysis of patients with SLE followed at five Rheumatology Departments between 1976 and 2006. Demographic data, diseases manifestations, medications used, co morbidity and damage scores were recorded. RESULTS: Five hundred forty four patients were studied, 93% female, 89% Caucasians, with an average age at disease diagnosis of 35 years. The most frequent clinical features were musculoskeletal (91%), cutaneous and mucous membrane (90%) and the hematological involvement (58%). Renal diseases and serositis occurred more often in males while myositis was more common in black patients. Immunological features included the presence of anti-nuclear antibodies in 99% of the patients, anti-DNA (76%) anti-SSA (33%), anti-SSB (20%), anti-RNP (26%), anti-Sm (22%), anticardiolipine (31%) and lupus anticoagulant (21%). Anti-SSA, anti-RNP, and anti-Sm antibodies were significantly more prevalent among black patients. The presence and severity of damage measured by SLICC/ACR was similar between sexes and ethnicities. In multivariate analyses diseases duration and the presence of hypertension showed a positive association, while educational and antimalarials were negatively associated with the presence of damage. CONCLUSION: In this cohort of Portuguese patients SLE present clinical features similar to those observed in other predominantly Caucasian populations, albeit a higher prevalence of anti-RNP and anti-Sm antibodies was observed. Some particular features were associated with male sex and African ethnicity. Some socio-demographic and clinical variables were associated with damage accrual.

[Osteopoikilosis: 2 clinical cases]. / Osteopoiquilose: dois casos clinícos.

Osteopoikilosis is a rare, benign and autosomal dominant bone disease. It is usually asymptomatic and diagnosed as a radiological finding. Plain X- Ray films show multiple sclerotic lesions on periarticular areas, epiphyses and metaphyses of long tubular bones. The authors describe two cases of osteopoikilosis in subjects belonging to the same family (brothers).

[Back pain and dorsal kyphosis in childhood]. / Raquialgias e cifose dorsal na infância.

Juvenile idiopathic osteoporosis (JIO) is a rare condition of unknown aetiology, with pre-pubertal onset and frequently spontaneous remission after puberty. We report a case of a 14 years old boy, which two years before began dorso-lumbar pain with dorsal kyphosis. At the age of 12, he was on percentil 25 for height and had no other symptoms or alterations on physical exam. He had multiple vertebral fractures, a low serum vitamin D, and a Z-score in lumbar spine of -5,3. Diagnosis of JIO was made after excluding other causes of juvenile osteoporosis. He was submitted to pamidronate therapy and after six months showed clinical and bone mineral density improvement. At the age of 14 he is asymptomatic. The authors present this clinical case because of is rarity and to point out that although many cases have spontaneous remission, without any therapy, some may persist and become more serious, with pain and multiple fractures, justifying therapeutic intervention.

Exercise stress echocardiography for detection of pulmonary arterial hypertension in a patient with systemic sclerosis.

The authors report the case of a 57-year-old patient, diagnosed with systemic sclerosis 16 years ago. She had been followed in the rheumatology outpatient clinic since 2003 and complained of marked fatigue and dyspnea on exertion. In addition to full medical evaluation, she also underwent standard Doppler echocardiography at rest, which was unremarkable, and exercise stress echocardiography. In the latter examination, she was diagnosed with pulmonary arterial hypertension, later confirmed during right heart catheterization. The authors discuss the potential value of this methodology for the study of patients with systemic sclerosis.