Characteristics of vascular involvement in Behçet's disease.

OBJECTIVE: Behçet's disease (BD) is a multisystemic inflammatory disorder classified among the vasculitides, which can affect all types and sizes of blood vessels. Vascular involvement may be seen in 25-50% of BD patients. In this study, we examined the characteristics of vascular involvement in patients with BD. METHODS: One hundred and eighty patients with BD were included in the study. The diagnosis of vascular involvement was made on clinical signs, by Doppler ultrasonography and/or angiography using computed tomographic or magnetic resonance techniques where appropriate. Detailed clinical characteristics were recorded for each patient. RESULTS: Seventy-one patients (39.4%) had vascular involvement. In patients with vascular lesions, the frequency of male sex was significantly higher than in patients without vascular lesions (89.8% vs. 63.3%, respectively; p < 0.001). Of 71 BD patients with vascular involvement, 68 had venous lesions (95.8%). Three patients had arterial lesions without venous thrombosis. Eleven patients had arterial involvement with venous thrombosis. The most frequent type of vascular involvement was deep venous thrombosis in the lower extremities (n = 56, 78.9%). There was a significant association between deep venous thrombosis and superficial thrombophlebitis (r = 0.325, p < 0.01). Twenty-four patients (33.8%) had vena cava thrombosis and two had vena hepatica thrombosis. In patients with vascular involvement, the frequency of erythema nodosum was significantly higher (p = 0.001) and the frequency of ocular involvement was significantly lower (p < 0.05) than in patients without vascular involvement. CONCLUSION: Our study illustrates the frequency and significance of vascular involvement in BD.

Association of psoriasis vulgaris with HLA class I and class II antigens in the Turkish population, according to the age at onset.

BACKGROUND: Association of psoriasis vulgaris with HLA antigens reference to age at onset has been reported in different racial or ethnic populations. OBJECTIVE: Our purpose was to determine the distribution of HLA markers in the Turkish population according to the age at onset of the psoriasis vulgaris. METHODS: HLA class I and class II antigens were performed by serologic methods in a group of 100 Turkish patients with psoriasis and 201 control subjects. Patients with psoriasis were subdivided into two groups based on age at onset (below or above 40 years of age) and family history. RESULTS: The frequency of HLA A30, Cw3, Cw6, DR7, DR14, DQ8, and DQ9 antigens were significantly increased in the Turkish psoriatic patients whereas HLA A66, Cw2, Cw4 and DR11 were found to be negatively associated with psoriasis. However, there were striking differences in HLA antigens according to the age at onset of the disease. Type I, early onset was associated with a high frequency of A30, B50, Cw6 and DR7 antigens whereas patients with type II, late onset had an increased frequency of Cw7. CONCLUSIONS: We conclude that psoriasis is probably a genetically determined disease and suggest that HLA-Cw6 antigen seems to associate commonly with early onset of psoriasis in Turkish patients.

Transforming growth factor beta-1 stimulates articular chondrocyte elaboration of matrix vesicles capable of greater calcium pyrophosphate precipitation.

Objective To determine the role of transforming growth factor beta1 (TGFbeta) in early calcium pyrophosphate formation by measuring its effects on articular chondrocyte matrix vesicle (MV) formation, specific activity of the inorganic pyrophosphate(PPi)-generating enzyme nucleoside triphosphate pyrophospho-hydrolase (NTPPPH) and biomineralization capacity. Methods MV elaborated from mature porcine chondrocyte monolayers+/-TGFbeta were compared for protein content, NTPPPH activity, and ATP-dependent biomineralization. Precipitation of calcium pyrophosphate mineral phases by MV was determined by a radiometric assay and by Fourier transform infrared spectroscopy (FTIR). Results MV from monolayers exposed to TGFbeta were enriched in NTPPPH activity compared to MV from control monolayers (P< 0.01) and precipitated more calcium/mg MV protein than controls (P

Systemic amyloidosis and sacroiliitis in a patient with systemic lupus erythematosus.

We report a case of a 25-year-old female with juvenile onset systemic lupus erythematosus who developed systemic secondary amyloidosis with renal and gastrointestinal involvement. She has also had radiological signs of bilateral asymptomatic sacroiliitis without lower back pain or HLA-B27 antigen.

Image-directed color Doppler ultrasonography of kidney in systemic lupus nephritis.

We evaluated intrarenal arterial waveforms with image-directed color Doppler ultrasonography in 21 patients with systemic lupus erythematosus (SLE). The systolic-diastolic ratio, resistive index, and pulsatility index were compared with serum creatinine levels, creatinine clearance, and quantitation of urinary protein excretion, as well as with histopathologic scores of specimens obtained from 9 patients who underwent renal biopsy. Doppler parameters were in the normal ranges in all patients, without showing significant correlation with any of the histopathologic scores or laboratory parameters except creatinine levels. We conclude that image-directed color Doppler ultrasonography is of no practical value in the evaluation of lupus nephritis during the early stages of the disease.

Insulin-like growth factor-1 suppresses pyrophosphate elaboration by transforming growth factor beta1-stimulated chondrocytes and cartilage.

Our objective was to examine the effect of insulin-like growth factor-1 (IGF-1) on extracellular pyrophosphate (ePPi) elaboration by porcine cartilage. These studies further define the factors influencing ePPi accrual, a key step in calcium pyrophosphate dihydrate (CPPD) crystal formation. ePPi was measured in adult porcine organ and monolayer culture media in the presence of IGF-1, transforming growth factor beta-1 (TGFbeta-1), IGF-1 antibody and synovial fluid (SF). As previously shown, TGFbeta-1 stimulated ePPi elaboration by cartilage and chondrocytes. IGF-1 significantly inhibited the stimulatory effect of TGFbeta-1 on ePPi elaboration by both cartilage explants and chondrocytes. Anti-IGF-1 antibody blocked this inhibition. Anti-IGF-1 antibody also decreased the inhibitory effect of SF on ePPi elaboration, suggesting the presence of active IGF-1. These results support an important regulatory role for IGF-1 in cartilage ePPi elaboration. IGF-1 inhibited the effects of the ePPi-stimulatory factor TGFbeta-1 and thus may protect normal joints from excess accumulation of ePPi and subsequent CPPD crystal formation.

Association of HLA class I and class II antigens with rheumatic fever in a Turkish population.

The distribution of class I and class II HLA antigens of 100 Turkish patients with rheumatic fever, 77 of whom had cardiac involvement, was examined. We compared the results with a control group of identical origin. The frequency of HLA A10 and HLA B35 antigens were found significantly higher in patients with rheumatic fever (p < 0.05, p < 0.01, respectively). The frequency of HLA A10 and HLA DRw11 in patients with cardiac involvement were significantly higher than in those without cardiac involvement (p < 0.05, p < 0.01, respectively). On the other hand, HLA Cw2 antigen frequency was found significantly higher in patients without cardiac involvement than in those with rheumatic heart disease (p < 0.05). We support the concept that rheumatic fever is an immunological reaction to group A, beta hemolytic streptococci in individuals who have genetic predisposition.

C3 activation products, C3 containing immune complexes, the terminal complement complex and native C9 in patients with rheumatoid arthritis.

Complement activation products, C9 and C3-containing circulating immune complexes (CIC), were evaluated in plasma and synovial fluid (SF) from patients with rheumatoid arthritis (RA) and osteoarthritis. C3 activation products and the fluid phase terminal complement complex were considerably elevated in SF from RA patients reaching levels five- to eighttimes that in plasma, consistant with a local activation of the whole cascade in the joints. The results emphazise the importance of detecting C3 activation by neoepitope expression instead of single fragment determinations. The concentration of native C9 was lower in synovial fluid compared with plasma, consistant with the excessive local complement activation. Increased CIC levels which correlated with the degree of complement activation were also found in the SF from the RA patients.

The leukocyte protein L1 in plasma and synovial fluid from patients with rheumatoid arthritis and osteoarthritis.

L1 is a major granulocyte and monocyte protein, released during activation and turnover of such cells. Blood and synovial fluid (SF) from 41 patients with rheumatoid arthritis (RA) and 6 patients with osteoarthritis (OA), were analyzed for L1 and the acute phase proteins C-reactive protein, orosomucoid, haptoglobin, alpha 1-antitrypsin and albumin as well as for differential leukocyte count. L1 levels in plasma and SF showed highly significant differences (p less than 0.0001), between the RA and OA patients. All the OA patients had normal plasma concentrations of L1 and low concentrations of L1 in SF. All the RA patients had elevated plasma levels of L1 and high L1 concentrations in SF. In the RA patients, the ratios between the protein concentrations in SF and blood were 3.29 for L1 and less than or equal to 0.64 for the acute phase proteins. In the SF, the L1 levels did not correlate with the monocyte count, while a low, positive correlation was found between L1 and the granulocyte count. The high L1 concentrations observed in SF from RA patients probably reflected an increased turnover of leukocytes in the inflamed joints. In SF from RA patients, high L1 concentrations were found in joints with a high amount of swelling. The present study suggests that L1 may represent a marker of both local and systemic inflammation.