Juvenile-onset Takayasu arteritis: peculiar vascular involvement and more refractory disease.

OBJECTIVES: To compare prognosis parameters and arterial site involvement in Takayasu arteritis (TA) patients with disease onset at age ≤ 18 and ≥ 21 years. METHODS: Sixty-two TA patients [American College of Rheumatology (ACR) and European League Against Rheumatism/Paediatric Rheumatology European Society (EULAR/PreS) criteria] were enrolled consecutively and divided into two groups according to disease onset, and matched for disease duration: juvenile TA patients aged ≤ 18 years (n = 17) and adult TA patients aged ≥ 21 years (n = 45). The protocol evaluated the following prognostic factors: aortic insufficiency, ischaemic retinopathy, severe systemic hypertension, and arterial aneurysms. In addition, death and remission [defined as stable disease > 6 months (no complaints without immunosuppressive and prednisone use) and normal erythrocyte sedimentation rate (ESR)] were also analysed. Stenosis and aneurisms were investigated by magnetic angioresonance or arteriography and angiographic classification was defined according to Hata criteria. RESULTS: Mean disease duration was similar in the juvenile and adult TA groups (13.50 ± 10.73 vs. 13.80 ± 7.17 years, p = 0.092) and a trend to a lower predominance of female gender in the juvenile TA group was observed (64.71% vs. 88.89%, p = 0.056). The prognosis was distinct in the two groups, with juvenile patients having a lower frequency of disease remission (23.53% vs. 55.56%, p = 0.04) and a significantly higher frequency of aneurism (41.0% vs. 11.1%, p = 0.013). Almost half of the juvenile TA patients had left renal stenosis, a frequency significantly higher than in the adult TA group (41.18% vs. 11.10%, p = 0.013), whereas the stenosis frequency was comparable in all other vascular sites evaluated. No differences were observed between the two groups regarding the frequency of aortic insufficiency, ischaemic retinopathy, severe systemic arterial hypertension, vascular procedures, and mortality. Angiographic classification revealed a similar distribution of arterial involvement in both groups (p > 0.05). CONCLUSIONS: Juvenile TA patients have distinct characteristics, with a peculiar renal vascular involvement, the presence of aneurism, and a more refractory disease compared with adult TA patients.

Demographic, clinical, and angiographic data of patients with Takayasu arteritis in Brazil.

We collected clinical, demographic, and angiographic data of Takayasu arteritis (TA) patients followed at Rheumatology Division of three Public University Centers (UNIFESP, USP, and UNICAMP) located in São Paulo State, Brazil. Clinical and demographic data were obtained from 73 patients (61 female; 50 white) The mean age at time of diagnosis was 27 y.o. and the mean follow-up time was 5 years. The following clinical features were observed along the evolution of these patients: absent or reduced pulses in upper limbs (85%), arterial bruit (64.5%), claudication of upper members (57%), headache (45%), hypertension (35.5%), dizziness (29%), cardiac bruit (29%), weight loss (27.5%), arthralgia or arthritis (26%), fever (24.5%) and claudication of lower limbs (20.5%). According to new angiographic classification of Takayasu arteritis performed in 28 patients followed at UNTFESP, with routine full aortography, 21% were classified as type I, 4% as type IIa, 4% as type III, 14% as type IV and 57% as type V. No patients had type IIb. Patients with Takayasu arteritis in São Paulo State Brazil showed female predominance and arterial involvement pattern similar to the one observed in Japan, with more than 20% classified as type I with exclusive involvement of the branches from the aortic arch.

[Subcutaneous phaeohyphomycose due to Exophiala jeanselmir. Report of 3 cases in patients with a kidney transplant]. / Feo-hifomicose subcutânea por Exophiala jeanselmei. Registro de três casos em transplantados renais.

We report three cases of subcutaneous phaeohyphomycosis due to Exophiala jeanselmei (Langeron) McGinnis et Padhye 1977, in kidney transplant patients. Exophiala jeanselmei is a dematious fungus having also ability to rarely procedure eumycetoma (black grains). According to KWON-CHUNG & BENNETT (1992) such fungus is antigenically very heterogeneous, since so far three serotypes have been identified; each serotype including subgroups. Subcutaneous phaeohyphomycosis is becoming more and more frequent in kidney transplant patients submitted to an immunosuppressive treatment. As Exophiala jeanselmei has already been isolated from the environment it becomes difficult to explain the pathogenicity of these cases by a reactivation of quiescent processes. The authors suggest an occasional fungistatic action of cyclosporine A upon Exophiala jeanselmei.

[Systemic lupus erythematosus in the aged: clinical and laboratory characteristics]. / Lúpus eritematoso sistêmico no idoso: características clínicas e laboratoriais.

The clinical and serologic characteristics of 199 systemic lupus erythematosus patients with early and late onset of disease were compared to determine if the disease in the older age group defines a specific subset of SLE. This study demonstrated that SLE in the elderly patients exhibits peculiar clinical features with a high frequency of muscular involvement (p < 0.05) and low frequency of cutaneous manifestations (p < 0.001) and alopecia (p < 0.02). Moreover, the most frequent clinical manifestation was muscular pain and stiffness, arthritis and weight loss (over 10 kg). This condition is often hard to distinguish from polymyalgia rheumatica or underlying malignancy. The frequency of autoantibodies was similar in both groups. The absence of anti-La was surprising, however it was confirmed by "Western blotting". The symptoms of late onset SLE are not very prominent however the diagnosis should be considered in order to avoid delays in treatment.

Antibodies to new cytoplasmic autoantigens: anti-JA, a potential marker for disease activity in systemic lupus erythematosus.

1. We describe new autoantibodies which recognize two cytoplasmic proteins of 30 and 26 kDa. They were detected by Western blot analysis in the sera of 6 of 79 randomly selected systemic lupus erythematosus (SLE) patients and are denoted anti-JA antibodies. This antibody specificity is different from the previously described lupus autoantibodies, anti-P and anti-S10. 2. The targeted autoantigens are trypsin sensitive, and resistant to RNase and DNase treatment. The binding to the antigens was not modified when reticulocyte ribosomes were prepared with protease inhibitors indicating that these are primary antigens and not degradation products. Several lines of evidence suggest that these proteins are almost certainly part of the ribosome. 3. Anti-JA reactivity was not observed in the sera from 60 patients with other autoimmune diseases or from normal individuals. In contrast, 55% of lupus sera selected for a high titer of anti-dsDNA (double stranded DNA) and LE cells were also anti-JA positive. 4. Anti-JA antibodies may be useful as a specific serological marker for disease activity in SLE. The strong association with anti-dsDNA antibodies and LE cell in the sera of SLE patients requires further study.

Antibodies to new cytoplasmic autoantigens: anti-ja, a potential marker for disease activity in systemic lupus erythematosus

We describe new autoantibodies which recognize two cytoplasmic proteins of 30 and 26 kDa. They were detected by Western blot analysis in the sera of 6 of 79 randomly selected systemic lupus erithematosus (SLE) patients and are denoted anti-JA antibodies. This antibody specificity is different from the previously described lupus autoantibodies, anti-P and anti-S10. The targeted autoantigens are trypsin sensitive, and resistant to RNase and DNase treatment. The binding to the antigens was not modified when reticulocyte ribosomes were prepared with protease inhibitors indicating that these are primary antigen and not degradation products. Several lines of evidence suggest that these proteins are almost certainly part of the ribosome. Anti-JA reactivity was not observed in the sera from 60 patients with other autoimmune diseases or from normal individulas. In contrast, 55% of lupus sera selected for a high titer of anti-ds DNA (double stranded DNA) and LE cells were also anti-JA positive. Anti-JA antibodies may be useful as a specific serological marker for disease activity in SLE. The strong association with anbti-ds-DNA antibodies and LE cell in the sera of SLE patients requires further study

[Reactive arthritis associated with infections and intestinal infestations]. / Artrites reacionais associadas a infecções e infestações intestinais.

Reactive arthritis is a non suppurative articular inflammation following an infection elsewhere in the body. It probably occurs by interaction of external infections agents and immunological factors that mediate the host's response to the agent's antigens. The authors reviewed the role of gastrointestinal infection and infestation as a cause of reactive arthritis. Since there are no good diagnostic criteria for reactive arthritis and the investigations in this area are difficult, it is possible that many of these cases are misdiagnosed.

[Pancreatitis and hepatitis associated with systemic lupus erythematosus]. / Pancreatite e hepatite associadas ao lúpus eritematoso sistêmico.

A patient with systemic lupus erythematosus presenting abdominal pain, nausea, vomiting and severe mucocutaneous vasculitis had significant elevation of serum amylase and hepatic enzymes levels during a flare-up of the disease. Clinical and laboratory alterations disappeared after therapeutic increase of corticosteroids given to the patient a fact that suggested etiologic correlation between systemic lupus erythematosus, hepatitis and pancreatitis.