[Show abstract][Hide abstract] ABSTRACT: Diffuse alveolar hemorrhage (DAH) is a rare but potentially catastrophic manifestation with a high mortality. Among rheumatologic diseases, it occurs most frequently in patients with systemic lupus erythematosus (SLE) and systemic vasculitis. Despite new diagnostic tools and therapies, it remains a diagnostic and therapeutic challenge. The aim of this work was to characterize the SLE patients with an episode of alveolar hemorrhage followed in our Clinical Immunology Unit (CIU). A retrospective chart review was carried out for all patients with SLE followed in CIU between 1984 and the end of 2013. We reviewed the following data: demographic characteristics, clinical and laboratory data, radiologic investigations, histologic studies, treatment, and outcome. We identified 10 episodes of DAH, corresponding to seven patients, all female. These represent 1.6% of SLE patients followed in our Unit. The age at DAH attack was 42.75 ± 18.9 years. The average time between diagnosis of SLE and the onset of DAH was 7.1 years. Three patients had the diagnosis of SLE and the DAH attack at the same time. Disease activity according to SLEDAI was high, ranging from 15 to 41. All patients were treated with methylprednisolone, 37.5% cyclophosphamide and 28.6% plasmapheresis. The overall mortality rate was 28.6%.
[Show abstract][Hide abstract] ABSTRACT: Objective
Behcet's disease (BD) is a chronic, relapsing, inflammatory vascular disease with no pathognomonic test. Low sensitivity of the currently applied International Study Group (ISG) clinical diagnostic criteria led to their reassessment. Methods
An International Team for the Revision of the International Criteria for BD (from 27 countries) submitted data from 2556 clinically diagnosed BD patients and 1163 controls with BD-mimicking diseases or presenting at least one major BD sign. These were randomly divided into training and validation sets. Logistic regression, leave-one-country-out' cross-validation and clinical judgement were employed to develop new International Criteria for BD (ICBD) with the training data. Existing and new criteria were tested for their performance in the validation set. ResultsFor the ICBD, ocular lesions, oral aphthosis and genital aphthosis are each assigned 2 points, while skin lesions, central nervous system involvement and vascular manifestations 1 point each. The pathergy test, when used, was assigned 1 point. A patient scoring 4 points is classified as having BD. In the training set, 93.9% sensitivity and 92.1% specificity were assessed compared with 81.2% sensitivity and 95.9% specificity for the ISG criteria. In the validation set, ICBD demonstrated an unbiased estimate of sensitivity of 94.8% (95% CI: 93.4-95.9%), considerably higher than that of the ISG criteria (85.0%). Specificity (90.5%, 95% CI: 87.9-92.8%) was lower than that of the ISG-criteria (96.0%), yet still reasonably high. For countries with at least 90%-of-cases and controls having a pathergy test, adding 1 point for pathergy test increased the estimate of sensitivity from 95.5% to 98.5%, while barely reducing specificity from 92.1% to 91.6%. Conclusion
The new proposed criteria derived from multinational data exhibits much improved sensitivity over the ISG criteria while maintaining reasonable specificity. It is proposed that the ICBD criteria to be adopted both as a guide for diagnosis and classification of BD.
Journal of the European Academy of Dermatology and Venereology 02/2013; 28(3). DOI:10.1111/jdv.12107 · 2.83 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Human leukocyte antigen (HLA)-B*51 is a well-known genetic factor associated with Behçet's disease (BD). To analyse the influence of HLA-B*51 and other HLA class I alleles in BD susceptibility in a Portuguese population and its association with disease severity, we studied 78 BD patients and 208 healthy controls. The patients were classified into two severity groups as described by Gul et al. As expected, a higher frequency of HLA-B*51 was found. The frequency of HLA-Cw*16 alleles was significantly higher in patients. Regarding severity, HLA-B*27 frequency was higher in the severe group compared with controls and with the mild group. Thus, HLA-B*51 and HLA-Cw*16 seem to confer susceptibility to BD in this patients. HLA-B*27 may be important as a prognostic factor.
[Show abstract][Hide abstract] ABSTRACT: Introdução: Os abcessos hepáticos constituem uma entidade clínica pouco frequente que coloca desafios no diagnóstico e tratamento. Nas últimas décadas assistiu-se a mudanças na epidemiologia, avanços nos meios de diagnóstico e ao aparecimento de mais alternativas terapêuticas.
[Show abstract][Hide abstract] ABSTRACT: Background: Behçet Disease (BD) is a rare multisystemic inflammatory disorder of unknown aetiology. Neurological involvement, currently known as Neuro-Behçet (NB) has been reported in the range of 2,2 to 50% in large series. Patients and Methods: From a database of 77 patients with BD, (all fulfilling the International Study Group criteria for the Diagnosis of BD, 1990), we identified 15 patients with Neuro-Behçet disease (NB). Clinical features and neuroimaging data were reviewed. Results: The frequency of NB in our centre was 19,5%. Gender frequency was similar: 8 males, 7 females (M/F: 1,11). HLA-B51 allele was present in 9 of 14 patients tested (64.3%). Mean age at onset of BD and at diagnosis was 29,6 and 34,3 years respectively, and the mean time between the first symptom and the central nervous system (CNS) involvement was 6,2 years. We divided patients in two distinct patterns of first neurological presentation: parenchymal - 9 patients (60%) - brain stem or brainstem plus 67%, hemispheric presentation 33% - and non-parenchymal - 6 patients (40%) - 4 aseptic meningitis, 1 optic neuropathy and 1 dural sinus thrombosis. MRI showed parenchymal lesions in 14 and non-parenchymal lesions in 1 patient. Mean follow-up was 7,8 years; 14 patients had a relapsing-remitting course and one a secondarily progressive course. Two patients were deceased with neurological complications and the others had no major sequels. Conclusion: The frequency of NB in our centre and clinical and neuroimaging features are similar to those reported in other series. Neurological involvement is an important cause of morbidity and mortality in these patients.
[Show abstract][Hide abstract] ABSTRACT: Lupus nephritis (LN) is a frequent and serious manifestation of systemic lupus erythematosus. However, the outcome has progressively improved in the last 3 decades and this was due to more efficient and early treatment of LN and comorbid situations. The aim of our study was to analyze our experience and outcome in LN, to evaluate clinicopathologic and clinicolaboratory correlations and to search for risk factors for renal and patient survival.
We conducted a retrospective study of 78 patients with biopsy-proven LN.
Acute renal failure and subnephrotic proteinuria with microhematuria occurred each one in 39.7% of the patients, nephrotic syndrome in 16.7% and nephritic syndrome in 3.8%. The mean serum creatinine at presentation was 1.45 +/- 1.03 mg/dl and the creatinine clearance was 68.2 +/- 40.3 ml/min. Class IV LN existed in 71.8%, Class III in 20.5%, Class V in 6.4% and Class VI in 1.3%. The treatment included steroids and cytotoxic agents in 87.5% of the patients with proliferative LN. Hypertension, serum creatinine and acute renal failure at presentation, as well as significant chronicity on renal biopsy, were significantly correlated with the progression to chronic renal failure in our population. Males were more prone to develop renal flares. 3.8% of the patients died, 9% lost their renal function, 26.9% are in remission, 33.3% still have subnephrotic proteinuria and microhematuria, 7.7% have nephrotic syndrome and 19.2% have chronic renal failure. The mean global follow-up was 102 +/- 74 months and 96.2% of the patients survived. The actuarial renal survival was 96.1% in the first year; 89.9% at 5 years; and 83.7% at 10 and 20 years.
We can say that hypertension, serum creatinine and acute renal failure at the onset and significant chronicity on renal biopsy, proved to be risk factors for chronic renal failure in our study population. Male gender was a risk factor for renal flares. The achieved global outcome can be considered a good result.
[Show abstract][Hide abstract] ABSTRACT: Uveitis is a general term that refers to the inflammation of uveal tract, which is an important cause of blindness in young people. It is well known that uveitis can be the initial manifestation of a systemic disease (S.D.), and may appear years before the diagnosis of the primary disease. Uveitis should be integrated in a systemic study with proper testing. Therefore, the diagnosis is a matter for the ophthalmologist and the Specialist in internal medicine. We have made a retrospective study of 71 patients with chronic uveitis or panuveitis. We found 54.9% of primary uveitis and 45.1% of S.D. associated uveitis, most of them with Behçet's disease (16/71) and Ankylosing Spondilytis (7/71). HLA typing of the patients showed a decreased frequency of HLA A1 and HLA A3 antigens and an increased frequency of the HLA B27 antigen, when compared to a Portuguese control population. We confirmed the important role of HLA B27 as an independent susceptibility factor for anterior uveitis. The lowest HLA A3 frequency was observed in the group of S.D. associated uveitis, which could suggest that this antigen may play a role as a factor of resistance to uveitis.
[Show abstract][Hide abstract] ABSTRACT: We report a case of a 23 year-old man admitted to hospital with cough, fever and recurrent episodes of haemoptysis. Laboratory findings, which included a pulmonary biopsy, established the diagnosis of Idiophatic Pulmonary Hemosiderosis (IPH). Despite a number of morphologic, immunologic and ultrastructural studies, the etiology and pathogenesis of this disease remain indetermined. To diagnose an IPH all the other causes of pulmonary hemorrhage must be excluded. This is a rare disease, even more rare in adults. Based on these facts the authors publish this article, which includes the case report and a literature review.