[show abstract][hide abstract] ABSTRACT: The authors review the practical aspects of biological therapy use for rheumatoid arthritis patients, commenting safety issues before and after treatment initiation and the best treatment strategies to optimize efficacy.
[show abstract][hide abstract] ABSTRACT: The creation of a physician-administered questionnaire to screen patients with Systemic Lupus Erythematosus (SLE) for the presence of symptoms suggestive of neuropsychiatric involvement (NPSLE).
The development of the questionnaire followed three phases. First, a list of manifestations was prepared based on the ACR case definitions for NPSLE. A first questionnaire was constructed including 119 items. To reduce their number, a Delphi analysis was carried out and a second questionnaire with 62 questions was developed. This questionnaire was administered to 139 patients with SLE (58 with NPSLE: 29 active, 29 inactive; and 81 without NPSLE: 39 active, 42 inactive). Questions relevant to the screening of patients were selected on the basis of the receiver operating characteristic (ROC) curve analysis.
Twenty-seven questions concerning central nervous system and psychiatric manifestations were found to be relevant; the remaining could be eliminated without significantly affecting AUC. The area under the ROC curve (AUC) was 0.69 (95% CI 0.61-0.78). A score above 17 was considered as suggestive of the presence of NPSLE with a sensitivity of 92.9% (95% CI 85.1-97.3 %) and specificity of 25.4% (95% CI 14.7-39.00 %).
This questionnaire could represent a 'core set' of questions that could help in clinical practice to identify patients with neuropsychiatric symptoms requiring further evaluation.
[show abstract][hide abstract] ABSTRACT: With the purpose of contributing to a better knowledge of the APCs functional activity in SLE, we evaluated the distribution and functional ability to produce pro-inflammatory cytokines (TNF-α, IL-1β, IL-6 and IL-12) of peripheral blood (PB) monocytes and DC (tDC), particularly myeloid (mDC) and CD14(-/low)CD16(+) DC subpopulations comparing them with those obtained from healthy individuals. The study was performed in 34 SLE patients with diverse disease activity scores (SLEDAI) and 13 healthy age- and sex-matched controls (NC). Our results show an overall decrease in absolute number and relative frequency of tDC in SLE patients with active disease when compared to those with inactive disease and NC, although this decrease did not seem to have an effect on the distribution of PB DC subsets. The monocytes number in SLE patients was similar to those found in NC, whereas a higher frequency of monocytes producing cytokines as well as the amount of each cytokine per cell found without stimulation was particularly observed in those patients with active disease. After stimulation, we observed a higher frequency of IL-12-producing monocytes in active SLE patients. On the other hand, we found among DCs higher frequencies of cytokine-producing CD14(-/low)CD16(+) DCs and a higher amount of cytokines produced per cell, particularly in active disease. These findings support an increased production of inflammatory cytokines by APCs in active SLE, mostly associated with alterations in CD14(-/low)CD16(+) DC subset homeostasis that might contribute to explain the dynamic role of these cells in disease pathogenesis.
Rheumatology International 01/2011; 32(4):863-9. · 2.21 Impact Factor
[show abstract][hide abstract] ABSTRACT: To compare frequency and functional activity of peripheral blood (PB) Th(c)17, Th(c)1 and Treg cells and the amount of type 2 cytokines mRNA we recruited SLE patients in active (n=15) and inactive disease (n=19) and healthy age- and gender-matched controls (n=15). The study of Th(c)17, Th(c)1 and Treg cells was done by flow cytometry and cytokine mRNA by real-time PCR. Compared to NC, SLE patients present an increased proportion of Th(c)17 cells, but with lower amounts of IL-17 per cell and also a decreased frequency of Treg, but with increased production of TGF-beta and FoxP3 mRNA. Iotan active compared to inactive SLE, there is a marked decreased in frequency of Th(c)1 cells, an increased production of type 2 cytokines mRNA and a distinct functional profile of Th(c)17 cells. Our findings suggest a functional disequilibrium of T-cell subsets in SLE which may contribute to the inflammatory process and disease pathogenesis.
[show abstract][hide abstract] ABSTRACT: The purpose of our study was to evaluate the frequency of leukocyte count abnormalities in a large cohort of SLE patients and its association with infection. To make this evaluation, we studied consecutive patients with SLE diagnosis and prospectively followed them in the Coimbra Lupus Cohort. Data about white blood cell count abnormalities and infection during one-year follow-up were obtained. The presence of leukopenia, lymphopenia, and neutropenia was registered for one-year period. Infections were classified as severe or mild. We found that of the 124 patients who were included (91.1% female, mean age 41.2, mean disease duration 11.1), mild infections occurred in 43.5%, and severe in 3.2% of the patients. Twelve percent, 41.1%, and 4.8% of the patients had persistent leukopenia, lymphopenia, and neutropenia, respectively. Fourteen percent received a pneumococcal vaccination. Patients with neutropenia had a significantly higher number of infections (P = 0.033). Thus, our study showed that neutropenia was associated with an increased risk of infection during this one-year follow-up. Infections were frequent, but most of them were mild.
Annals of the New York Academy of Sciences 09/2009; 1173:103-7. · 4.38 Impact Factor
[show abstract][hide abstract] ABSTRACT: The purpose of this study was to characterize the clinical and serological features of a large cohort of patients with antinuclear antibody (ANA) positive undifferentiated connective tissue disease (UCTD). Consecutive patients with UCTD, followed up at the Rheumatology Clinic of the participating centers, were included. Data from these patients were obtained by clinical evaluation and chart review. All patients were diagnosed as having UCTD on basis of the following criteria: positive ANA plus at least one clinical feature of connective tissue disease, but not fulfilling classification criteria for any differentiated connective tissue disease. One hundred eighty-four patients were studied (female patients-94.5%; mean age at time of evaluation-47 years). The most prevalent manifestations were arthralgia (66%), arthritis (32%), Raynaud's phenomenon (30%), sicca symptoms (30%), and leukopenia (19%). The prevalence of ANA was 100%, anti-SSA 20%, anti-dsDNA 14%, and anti-SSB 7%. Patients with anti-dsDNA/anti-Sm, anticentromere/anti-Scl70, or anti-SSA/anti-SSB antibodies more frequently presented a set of manifestations close to systemic lupus erythematosus (SLE), systemic sclerosis, or Sjögren syndrome, respectively. We analyze a large cohort of UCTD. Seventy-two percent of these UCTD patients present lupus-, scleroderma-, or Sjögren-like features but do not fulfill classification criteria and mostly present a mild disease.
[show abstract][hide abstract] ABSTRACT: Systemic Lupus Erythematosus is associated with an increased risk of atherosclerosis and cardiovascular events, as compared to the general population.
To evaluate the prevalence of cardiovascular risk factors in Portuguese SLE patients and to estimate the associated risk of cardiovascular events. To evaluate the needs for optimization of prophylactic measures to prevent cardiovascular events in these patients.
Consecutive SLE patients followed in the Coimbra Lupus Cohort were included. Traditional cardiovascular risk factors were evaluated and risk factors associated to the disease (renal disease, anti-phospholipid antibodies, therapy) were evaluated. The 10-year cardiovascular risk was estimated using the Framingham model. Institution or optimization of therapeutic measures to control cardiovascular risk factors was evaluated according to published preliminary guidelines.
102 SLE patients were included (female: 89.2%, mean age= 39.7 years old, mean disease duration=8.9 years). 40.5% are overweighted, obesity in 9.9%. 72.5% have sedentary lifestyle and 11.8% are currently smokers. 40.2% have arterial hypertension, 32% have dyslipidaemia. Metabolic syndrome was identified in 21% of patients. Of these patients, 79.05% present one or more cardiovascular classical risk factors. Were receiving more than 10 mg/day of prednisone, 23.6% of the patients. Estimated 10-year cardiovascular risk is higher than 1% in 24.7% patients. We estimate that 55.8% of patients might benefit from dietary changes, 79.4% from increase in physical activity, 55.9% could be on anti-hypertensive medication, 52,9% on lipid lowering therapy and 76.5% on low dose aspirin anti-platelet aggregation.
Similar to findings in other academic centers, the majority of our patients have at least one modifiable, traditional cardiovascular risk factor which had not been treated adequately by standard guidelines and possibly even detected. These measures could greatly improve the long-term prognosis of SLE patients.
[show abstract][hide abstract] ABSTRACT: Spondylodiscitis is a serious clinical entity. Despite the decrease in mortality from about 25% in the pre-antibiotic era to near 5%, it remains significant and the associated morbidity is still relevant. The rate of residual neurological deficits among survivors is around 7%. In 30% of patients some related symptoms persist, the most important being pain. The authors report the case of a 74-year-old male patient with recent onset low back pain, which caused considerable disability. With this work the authors intend to alert to the fact that in a patient with a common symptom such as low back pain, the presence of "red flags" requires a quick investigation and diagnosis in order to prevent serious damage.
[show abstract][hide abstract] ABSTRACT: Systemic Lupus Erythematosus (SLE) is a rheumatic autoimmune disease characterized by multisystemic involvement with a variable prognosis. The association with Antiphospholipid Syndrome (APS) occurs in about 36% of the patients, raising additional problems with treatment and monitoring of these patients. The authors report a clinical case of a girl with SLE and APS who represented Budd-Chiari Syndrome and severe thrombocytopenia. The patient had severe thrombotic event and simultaneously a high hemorrhagic risk due to thrombocytopenia. Long-term resolution of the thrombocytopenia was achieved with mycophenolate mofetil. Long-term anticoagulation for thrombosis prophylaxis is required.
[show abstract][hide abstract] ABSTRACT: To evaluate quality of life in Portuguese patients with Systemic Lupus Erithematosus (SLE) and its correlation with disease activity and cumulative damage.
We included consecutive SLE patients, fulfilling the 1997 ACR Classification Criteria for SLE and followed at the Rheumatology Department of the University Hospital of Coimbra, Portugal at time of visit to the outpatient clinic. Quality of life was evaluated using the patient self-assessment questionnaire Medical Outcomes Survey Short Form-36 (SF-36) (validated Portuguese version). The consulting rheumatologist fulfilled the SLE associated indexes for cumulative damage (Systemic Lupus International Collaborating Clinics- Damage Index: SLICC/ACR-DI) and disease activity (Systemic Lupus Erythematosus Disease Activity Index: SLEDAI 2000). Correlation between SLEDAI and SLICC and SF-36 was tested with the Spearman Coefficient. Significant level considered was 0.05.
The study included 133 SLE patients (90.2% female, mean age - 40.7 years, mean disease duration - 8.7 years). Most patients presented low disease activity (mean SLEDAI = 4.23) and limited cumulative damage (mean SLICC = 0.76). Despite that, SF-36 mean scores were below 70% in all eight domains of the index. Physical function domains showed lower scores than mental function domains. The QoL in this group of patients is significantly impaired when compared with the reference Portuguese population (p<0.05 in all domains). There was no correlation between clinical activity or cumulative damage and quality of life.
QoL is significantly compromised in this group of SLE patients, but not related with disease activity or damage. These findings suggest that disease activity, cumulative damage and QoL are independent outcome measures and should all be used to assess the full impact of disease in SLE patients.
[show abstract][hide abstract] ABSTRACT: The authors report a case of a 52-year old female previously followed at the Outpatient Rheumatology Clinic with the diagnosis of Fibromyalgia (FM). Approximately 2 years after this diagnosis, she presents with a 2nd degree burn in a hand, as a result of thermal hypoesthesia. The patient described hipostesia of the distal upper and lower limbs, incontinence of the anal sphincter and chronic diarrhoea with progressive worsening. The electromiography showed sensory-motor axonal polyneuropathy, chronic, moderate to severe. The muscle and nerve biopsy showed deposition of amyloid substance. The search for TTR Met 30 was positive, confirming the diagnosis of familial amyloidotic polyneuropathy. This is the first reported case of familial amyloidotic polyneuropathy as part of the differential diagnosis of fibromyalgia.
[show abstract][hide abstract] ABSTRACT: The authors present the case of a 44-year-old female patient with Systemic Lupus Erythematosus diagnosed 4 years earlier. She presented with constitutional symptoms and back pain and one month later a diagnosis of Non Hodgkin Lymphoma was established (subtype Diffuse Large B Cells Lymphoma). The risk of malignancy associated to SLE is discussed.
[show abstract][hide abstract] ABSTRACT: We report a case of severe type IV hypersensitivity reaction to amoxicillin, which occurred in a person with a 12-year history of SLE. The present case illustrates the wide differential diagnosis in a SLE patient who presents with an allergic drug reaction. The attribution of the presenting symptoms to the underlying SLE and/or to the drugs used to treat SLE and coexisting conditions is a major challenge.