The programmed cell death 1 gene 7209 C>T polymorphism is associated with the risk of systemic lupus erythematosus in the Polish population.

Numerous investigations indicated that the programmed cell death 1 (PDCD1) gene polymorphisms contribute to the development of systemic lupus erythematosus (SLE). However, their association with SLE has been found to be controversial. Therefore, in patients with SLE (n=102) and controls (n=140) we examined the association of six polymorphisms of this gene with susceptibility to SLE in the Polish population. We found that PDCD1 7209 CT or 7209 TT genotype exhibited 3.282-fold increased risk of SLE (95% CI=1.553 - 6.935; p=0.0017). The allele and genotype frequencies of the remaining polymorphisms: 5708 C>T, 6438 G>A, 7146 G>A and 8737 G>A did not exhibit statistical differences between SLE patients and controls. Our results confirmed the association of 7209 C>T polymorphism of PDCD1 gene with SLE that was previously observed in the Taiwanese population.

The effect of D-penicillamine on polymorphonuclear leukocyte function.

D-Penicillamine, a reducing and chelating agent used in the treating of rheumatoid arthritis, was tested for its effects of polymorphonuclear leukocyte chemotaxis, phagocytosis, and lysosomal enzymes. beta-Glucuronidase release from polymorphonuclear leukocytes after phagocytosis of latex particles was not affected by D-penicillamine at concentrations ranging from 25 to 400 mg/liter. No direct effect was seen on enzyme activity at the maximum concentration of the drug. There was no inhibition of latex particle ingestion. No cell damage was found at 400 mg/liter penicillamine as measured by lactic dehydrogenase release. At this drug concentration there was only a 15% reduction in hemolytic complement levels. Chemotaxis was significantly decreased at concentrations of 50 mg/liter with a dose-dependent effect at higher concentrations which showed a plateau from 200 to 400 mg/liter. The parent compound D-cysteine was also tested in these systems. The same lack of effect of phagocytosis and enzyme release was found. D-Cysteine did inhibit chemotaxis but to a lesser degree than D-penicillamine. This dicotomy of drug effect may indicate that the beneficial action of D-penicillamine in the treatment of rheumatoid arthritis is due to the decreased chemotaxis of polymorphonuclear leukocytes into the joint, while the absence of an effect of phagocytosis and lysosomal enzymes shows the cells can still function to ingest and destroy bacteria. This latter effect correlates with the absence of infection in patients treated with this compound.

Systemic lupus erythematosus in Europe at the change of the millennium: lessons from the "Euro-Lupus Project".

The "Euro-Lupus Cohort" is composed by 1000 patients with systemic lupus erythematosus (SLE) that have been followed prospectively since 1991. These patients have been gathered by a European consortium--the "Euro-Lupus Project Group". This consortium was originated as part of the network promoted by the "European Working Party on SLE", a working group created in 1990 in order to promote research in Europe on the different problems related to this disease. The "Euro-Lupus Cohort" provides an updated information on the SLE morbidity and mortality characteristics in the present decade as well as defines several clinical and immunological prognostic factors.

Evaluation of cerebrospinal fluid for the presense of anticardiolipin antibodies (aCL) in NP-SLE patients.

Many neurological or psychiatric manifestations of SLE (NP-SLE) are related to the presence of anticardiolipin antibodies (aCL) in the patient's sera. The aim of this study was to evaluate the presence of aCL in cerebrospinal fluid (CSF) in SLE patients with NP features. Fifteen SLE patients were studied, all with NP features. CSF was evaluated for intrathecal IgG synthesis, oligoclonal IgG, and blood-brain barrier impairment. Sera and CSF were tested by ELISA for the presence of aCL-IgG and aCL-IgM with and without beta2 glycoprotein (beta2 GPI) cofactor. CSF and sera of 50 low back pain patients served as controls. Six patients were aCL(+) and nine aCL(-). In all patients the general CSF examination was normal. In all patients the value of indices of intrathecal IgG synthesis were normal but oligoclonal protein was present in the CSF of three patients. In none of the patients was the blood-brain barrier impaired. Neither aCL-IgG nor aCL-IgM was detected in the CSF of any NP-SLE patient. Mean levels of aCL in patients without cofactor beta2 GPI and with cofactor were as follows: for IgG class 0.005 and 0.057 OD (negative); for IgM class 0.004 and 0.024 OD (negative). We could not detect aCL in the CSF of patients with NP-SLE, even if sera were positive for aCL.

[Variety of initial symptoms in patients with Wegener's granulomatosis]. / Róznorodnosc objawów poczatkowych u chorych z ziaminiakiem Wegenera.

Wegener's granulomatosis is a necrotizing vasculitis with heterogenous initial symptoms what may still cause diagnostic problems. We present analysis of initial symptoms of 14 patients with Wegener's granulomatosis to evaluate their value in early diagnosis. Involvement of kidneys as well as upper and lower airways may suggest the diagnosis. Acute onset with intensified general symptoms may provoke more intensive diagnostic procedures and the diagnosis can be established earlier. In patients with non-acute onset the diagnosis may be delay, in some cases a few years after the first symptoms.