Jornal de Vasculite

Behçet’s disease (BD) is an inflammatory disorder characterized by recurrent oral and genital ulcers, ocular inflammation, arthritis and skin lesions. Neuro-Behçet’s disease (NBD) is found in 5–30% of patients and is classified into parenchymal and non-parenchymal manifestations. Most common parenchymal NBD manifestation is brainstem meningoencephalitis and patients may also have cranial nerve palsies, myelitis, epilepsy and peripheral neuropathy. Non-parenchymal NBD manifestations are cerebral venous thrombosis and aseptic meningitis. NBD usually develop abruptly and generally clear completely within weeks, however a third of patients evolve with progressive course. Moreover, patients with BD without overt neurological manifestations may present silent neurological involvement, with abnormal findings on neuropsychological, neurophysiological and neuroimaging studies. NBD is an adverse prognostic factor. Herein we review NBD manifestations and its treatment.

Purpose: The aim of our study was to evaluate the role of multilayer stents (MLS) in the therapeutic arsenal of complex aortic pathologies for frail patients combining several comorbidities that are initially objected to the conventional surgical treatment.
Materials and methods: Since March 2012, we conducted a monocentric prospective study on a series of 41 procedures, performed on 41 aortic lesion locations in 38 patients treated with multilayer stents. The series was divided into four groups. The first group included 21 cases: 20 thoracoabdominal aneurysms and one aneurysm of the entire descending thoracic aorta. The second group included 7 cases of juxta/infra renal aneurysms, the third group included 5 cases of false aneurysms and the fourth group included 8 cases of aortic dissecting hematoma (first subgroup: 6 cases) or extended dissections (second subgroupe: 2 cases).Three patients were treated during the same operation for a double aneurysm location (thoracic and abdominal in 2 cases and aortic arch and thoracic aorta in the 3rd case).
Results: The series included 25 men and 13 women with an average age of 63 years (40-84 years). The procedures were performed under local anesthesia in 3 cases, loco regional in 19 cases and general anesthesia in 16 cases. The average hospital stay was 7 days (4-14 days).The technical placement of the MLS was successful for the 41 procedures. No paraplegia, no stroke and no mesenteric ischemia occurred. Three complications related to the surgical approach were treated surgically with success. Two patients had post-operative renal failure for which one required dialysis. The 30-day mortality in relation to the aneurysm was 3% (one case of rupture in the first postoperative day) and unrelated to the aneurysm 3% (one case of massive myocardial infarction after the procedure). For an average follow up of 12 months (1-20 months) mortality unrelated to the aneurysm was 25% (9 deaths) and 0% in relation to the aneurysm. Among the 38 remaining aortic lesions treated and followed, the aneurismal sac was not feeded in 14 cases (63%) with 6 out of 20 cases in group 1(30%), 3 out of 6 cases in group 2(50%), 3 out of 4 cases in group 3 (75%) and finally 2 out of 6 cases in the first subgroup of group 4 (33%). The aneurismal sac is not feeded in 19 cases (50 %), including 14 out of 20 cases in group 1 (70%), 2 out of 6 cases in group 2 (33%), 1 out of 4 cases in the group 3(25%) and 2 out of 6 cases in the first subgroup of group 4 (33%). Two cases of extensive dissections treated remained stable without aneurismal changes. Three cases are recent: 1 out of 6 cases in group 2 (17%) and 2 out of 6 (33%) in the first subgroup of group 4 (33%). Visceral arteries and supraaortic trunks remained patent in all the 38 cases.
Conclusion: MLS may be an option treatment for dissections and complex aortic aneurysms in frail patients with high surgical risk. Our results showed that better results are obtained for false aneurysms and true aortic aneurysms without involvement of significant side branches.

Objective: To describe the clinical, serological characteristics, and renal histology in Mexican patients with antineutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV).
Methods: Forty patients with diagnosis of AAV and renal biopsy, followed at a tertiary care center in Mexico City from 2002-2012 were retrospectively studied. Demographics, comorbidities, clinical, laboratory variables, disease activity, mortality, renal variables and histological classes were analyzed. Additionally, 50 patients with AAV, and a control group (other systemic vasculitis, systemic lupus erythematosus, infections, healthy subjects) were studied transversally to assess the prevalence of specific antibodies (ANCA, MPO, PR3, cathepsin G, lysozyme, lactoferrin, BPI and elastase), and their association with clinical manifestations.
Results: Granulomatosis with polyangiitis (GPA) was the predominant phenotype, and median age at diagnosis was 53 years. High disease activity (BVAS/GPA=24), general symptoms (98%), renal (80%), pulmonary (63%), and ear, nose and throat (ENT) (58%) involvement were frequent at diagnosis. Renal damage was present at diagnosis (serum creatinine 3.9 mg/dl, estimated glomerular filtration rate 16 ml/min, 24 hr proteinuria 2.5 g, 15% on dialysis). The most prevalent renal histological class was sclerotic (58%), followed by mixed (32%), focal (8%), and crescentic (2%). No significant differences were found between histological classes in comorbidities, clinical, renal and laboratory variables, or survival. The main ANCA immunofluorescence pattern was C-ANCA (68%), followed by XANCA (22%), and P-ANCA (18%). PR3-ANCA (high sensitive) was the most frequent antibody, positive in 86%, while positivity for MPO-ANCA was observed in 18%. ANCA positivity was more frequent in patients with AAV than the control group (p<0.001). Antibodies directed to minor targets were infrequent in patients with AAV (2%). No significant correlations were identified between autoantibodies and clinical manifestations.
Conclusion: GPA phenotype, C-ANCA and PR3-ANCA positivity, high disease activity, chronic renal damage, general symptoms, ENT and lung involvement at diagnosis distinguish our population with AAV.

Background and objective: Takayasu's arteritis is a disease that has been associated with tuberculosis, based upon the presence of Langerhans giant cells and granulomas. ATP treatment of mycobacteria infected macrophages induces apoptosis mediated via the P2X7 pathway. Here we evaluated if the polymorphisms of the P2X7 receptors are associated to the presence of tuberculosis in Mexican patients with Takayasu´s arteritis.
Methods: 63 aortic samples, 33 from Takayasu´s and 30 from tuberculosis subjects were studied. Histological analysis was made and the sequence of the gene IS6110 which identifies the presence of Mycobacterium tuberculosis was amplified. Polymorphisms of the A1513C and -762 C/T receptors for the P2X7 gene were analyzed.
Results: The analysis showed a high percentage of extra pulmonary lesions (36.3%) in the Takayasu´s group. No significant differences between the studied genotypes were found in any of the models analyzed or in any of the two P2X7 polymorphisms analyzed. Moreover, an increase in the 1513C allele in presence of IS6110 gene in the Takayasu´s group was observed when comparing with the group of tuberculosis.
Conclusion: We found a high percentage of extra pulmonary tuberculosis in subjects with Takayasu´s arteritis; this increase was associated with a high frequency in the allele 1513C in the presence of IS6110 gene. Thus, these alleles may confer a significant susceptibility at the aortic level in the Mexican population having these diseases.

Vasculitis is not an uncommon diagnosis, however ocular manifestations of vasculitis may at times be presenting symptoms of the disease, requiring an earnest effort on part of the ophthalmologist. In the eye, retinal vessels are the most susceptible to inflammation and sometimes, ocular signs may provide vital clues to the systemic etiology. This review of ocular manifestations of vasculitis is structured in accordance with latest Chapel Hill nomenclature encompassing both local and systemic forms of the disease. It is a synopsis of latest clinical manifestations and diagnostic criteria’s of vasculitis, with a summary on newer investigations and treatment guidelines. It attempts to provide an overview for not only ophthalmologists but also rheumatologist, as proper management of vasculitis requires a multi-disciplinary approach with urgent inter departmental referral that could at times be both sight and lifesaving for the patient.

Diffuse Subendocardial Vasculitis (DSV), either primary or secondary, may occur during autoimmune diseases and lead to high mortality and morbidity. Recently, Cardiovascular Magnetic Resonance (CMR), a non-invasive, nonradiating technique, capable to perform tissue characterization has been used in the evaluation of cardiac vasculitis in the autoimmune diseases. CMR by performing tissue characterisation can detect diffuse, subendocardial oedema during the acute phase of DSV. To assess oedema, T2STIR images, T2 mapping and native T1 mapping can be used. Furthermore, diffuse subendocardial fibrosis can be also detected in both acute and chronic DSV. For this purpose, Late Enhanced Images (LGE) should be taken 15 minutes after the intravenous injection of the paramagnetic contrast agent gadolinium. Diffuse, subendocardial scar can be quantified and expressed as percentage of LV mass and this index have an important clinical value together with the LV ejection fraction in both ischemic and nonischemic cardiomyopathy.
DSV can be identified by CMR in both acute and chronic autoimmune diseases. Acute DSV has the potential to be reversed completely by autoimmune and cardiac treatment modification. CMR is a powerful tool to early diagnose DSV and guide therapeutic decisions. However, further studies are needed to establish its impact in the patients’ risk stratification and treatment follow up.

Primary vasculitides, where blood vessels are the primary site of inflammation, with the exception of Henoch Schönlein purpura (HSP) and Kawasaki’s disease (KD) are rare in childhood but when present, cause significant morbidity and mortality. This review provides an update on classification and presentation of rare vasculitides in children, through considering our experience in three cases in context with the current literature. We have demonstrated the challenges to diagnosis and how diagnosis can easily be missed if vasculitides are not carefully considered and looked for. The introduction of classification criteria has been important to allow more research into rare paediatric vasculitides but development of diagnostic criteria may be useful. Vasculitides should be considered in all patients presenting with multi-system features.