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In our article is referred to the recognition of collagenosis in the demarcated whole district with 100,000 inhabitants. The occurrence of these diseases is lower than the occurrence of other rheumatic diseases. Our results as well as the observations of other authors confirm that the lupus erythematodes and the diffuse sclerodermia are not rare diseases even in our circumstances and with their evolution tendency they shorten the lives of the affected persons. The high recognition coefficient with large probability corresponds to the real incidence of these two diseases, which can be achieved only by an observation of many years and repeated controls of the correctness of the diagnosis.

Prolonged repeated analyses of sera from 98 patients with systemic lupus erythematosus (SLE) and 76 patients with similar diffuse diseases of connective tissue by a radioisotope membrane binding method for the assessment of antibodies against double-stranded ribonucleic acid (anti-ds-RNA) revealed that these antibodies are most frequently encountered in patients with the evolutive form of SLE (53%), but also in approximately one fifth of patients with non- evolutive SLE and in patients with similar diffuse diseases of connective tissue. Anti-ds-RNA are not linked with stages of clinical activity; their occurrence is on the whole independent on the occurence of antibodies against ds-DNA and nuclear ribonucleoprotein. There is a very close association with the incidence of antibodies against denatured DNA and a substantially small association with antibodies against acid nuclear antigen (Sm). The occurrence of anti-ds-RNA is a favourable prognostic sign, as in the majority of patients who had such antibodies at any time in their serum, later the disease can be suppressed by treatment, while patients who never had anti-ds-RNA in serum remain for a long time in the active stage of the disease.

A family with hereditary C2 deficiency was discovered in Czechoslovakia. The proband is a 47-year-old female with a SLE-like syndrome and zero activity of the classical complement pathway. Functional CH50, C1, C2, and C4 estimations for all family members revealed a homozygous C2 deficiency in both the proband and her elder sister, and several heterozygotic C2-deficient individuals. The defect segregates with haplo-type HLA A25, B18, DR2.

Data are presented demonstrating the prevention of spontaneous autoimmune disease in NZB/Swiss mice treated natural double-stranded RNA. The successful treatment of animals was followed both by improvement in clinical manifestations and by the migration inhibition test performed with DNA as antigen. The reasons for the successful employment of natural double-stranded RNA are thought to be in its physicochemical properties.

In NZB mice of different ages (2, 4, 8, 12, and 16 months) the positivity of the migration inhibition test performed with the ds-DNA in spleen explants was compared with the level of immunofluorescent antinuclear antibodies and the incidence of glomerulonephritis. The positivity of the MIT was apparent at 4 months of age, rose with ageing and decreased after 12 months of age. Production of ANA began later, mostly at 8 months of age, increased progressively with ageing, especially in female mice, and was followed by the development of glomerulonephritis which reached its peak at 16 months of age.

A correlation was made between the severity of clinical symptoms of SLE and the results of the migration inhibitory factor (MIF) production test. For this purpose 10 patients with various manifestations of SLE were examined by MIF production test before and after the immunosuppressive therapy. A good correlation between the actual clinical stage and the results of the MIF production test was found. The successful immunosuppressive therapy turned the positive MIF production into the negative one in most of the observed patients. The MIF production test is therefore recommended as a good complementary test for the estimation of the actual state of the SLE.

The simultaneous occurrence of interferon and migration inhibitory factor was found in sera from 9 patients with systemic lupus erythematosus (SLE). Their clinical picture was characterized by a moderate course of the disease. In 7 patients the inflammatory tendoarticular manifestations were accentuated. Glomerulonephritis was found only in 3 patients and was successfully suppressed by immunosuppressive therapy. In the peripheral blood of most of these patients, normal or moderately increased levels of active E-rosettes were found. Therefore we consider these SLE patients as a group with characteristic signs of activated cell-mediated immunity (lymphokine release, active E-rosettes). Although the reason for this activation remains unknown, we suppose that some concomitant bacterial infection, perhaps mycobacterial, may contribute to this immunological phenomenon.

Based on long-term observations the authors submit a categorization of primary (hereditary and solitary) articular chondrocalcinosis into three different sub-populations. Attention is drawn to the fact that the extent of the qualitative disorder of the articular cartilage, obviously conditioned genetically, is linked with the age factor and determines the quantitative differences of pyrophosphate arthropathy in primary chondrocalcinosis. In young age, as a rule in the third decade, severe polyarticular condrocalcinosis (first sub-population) develops which causes relatively soon invalidity, in middle age (5th and 6th decade) milder condrocalcinosis develops (second sub-population) which combines with extraarticular, tendinous and tissue calcifacations, and finally in advanced age oligoarticular chondrocalcinosis develops (third sub-population) which is usually associated with ankylosing hyperostosis of the spine. Articular chondrocalcinosis (CCA) which we described by this term as a special metabolic arthropathy which occurs in families and solitary and which we defined as a special nosological unit (35, 36,) has become generally known and firmly established in rheumatology. As ensues from numerous publications, primary (idiopathic) CCA which comprises the hereditary and solitary (sporadic) form is characterized by pyrophosphate arthropathy which develops on articular cartilages not damaged by another process (13, 25, 26, 37); on the other hand as secondary CCA we consider pyrophosphate arthropathies which are associated with metabolic, endocrine or other diseases (9, 30). The common sign of both basic forms of CCA is the presence of microcrystals of calcium pyrophosphate dihydrate (CaPD) in articular cartilages, synovial fluid, or other articular structures (capsules, tendons, ligaments), characterized originally by McCarty et al. (11, 18) and later by other authors (2, 23, 27, 32). In addition to semantic (terminological) problems there were also questions of the classification of CCA because, based on an analysis of major groups of patients, it was revealed that there is a varied picture of chondrocalcinosis or pseudogout (18, 19) or deposition disease (17). of calcium pyrophosphate dihydrate crystals. In our paper we are presenting our view on the primary form of CCA and submitting the characteristics of classification which ensued from more than 20 years observation of our group of patients, in particular based on the evaluation of the beginning of the clinical and X-ray manifestations and the further development of the disease.

The administration of sonicated fractions of f2 phage polyribonucleotides caused an increased weight loss and deterioration of the clinical state in female NZB mice. Discontinuance of the treatment resulted in an improvement of both the clinical state and the genetically determined autoimmune disorders of these mice. Some potential explanations of this effect are discussed.

During a period of 4 years urine samples from 54 patients with systemic lupus erythematosus (SLE) were repeatedly analysed for the activities of lactate dehydrogenase (LDH), leucine aminopeptidase (LAP) and alanine aminopeptidase (AAP). Increased urinary enzyme levels were consistently found in 8 patients with severe lupus nephritis and the nephrotic syndrome, as well as in 9 patients with chronic lupus nephritis resistant to therapy. A further group of 9 patients with lupus nephritis responded favourably to immunosuppressive therapy with arrest of kidney-damaging processes; a concomitant normalization of urinary enzyme levels was observed, giving an accurate reflection of the progression of the disease. Another 14 SLE patients showed raised enzyme levels preceding the development of clinical signs of nephropathy. The last 14 SLE patients displayed neither nephropathy nor altered enzyme activities. The determination of urinary enzyme activities is, therefore, considered to be a useful supplement to the routine biochemical analyses performed on the urine in cases of SLE.

Ketanserin was used in a randomized double-blind trial in 15 patients with Raynaud's phenomenon in progressive systemic sclerosis (PSS). Its effect on Raynaud's phenomenon was evaluated by IR-radiometry, Doppler ultrasound, nailfold capillaroscopy, frequency of finger ulcerations and patient complaints before and after a 3-month course of treatment with oral ketanserin in the dosage of 60 mg daily in the first month and 120 mg in the second and third months. Of the 8 patients treated with ketanserin, 5 showed improvement. In the other 2 patients with progression of skin sclerosis and multiorgan involvement, the peripheral vascular disorder was unchanged. Ketanserin treatment was discontinued in one patient owing to dizziness and anxiety. In one patient ketanserin was reduced to 60 mg daily because of fluid retention. There were no other adverse effects. In 7 control patients on placebo there was no significant improvement in Raynaud's phenomenon. Ketanserin, a selective, specific and pure antagonist of 5-hydroxytryptamine (serotonin) appears to be an effective agent in the treatment of Raynaud's phenomenon and digital ischaemic ulcers in PSS. Moreover, ketanserin could contribute to the understanding of the role of 5-hydroxytryptamine in PSS pathogenesis.

In 177 patients with lupus nephritis in the course of three decades three methods of immunotherapy were used the effect of which was evaluated retrospectively. When the criterium of the survival period was used, it was revealed that from a group of 66 patients treated only with glucocorticoids (mainly prednisone by the oral route) less than 60% survived five years the onset of lupus nephritis, only 33% survived 10 years. From a group of 81 patients treated with glucocorticoids and cytostatics (mostly cyclophosphamide by the oral route) 80% survived five years and 50% ten years, while in a group of 30 patients treated with glucocorticoids, cyclophosphamide and levamisole 90% survive five years and as many as 70% ten years from the onset of lupus nephritis. From the onset of SLE however as many as 80% survived ten years in all three analyzed groups, which provides evidence that lupus nephritis accounts for the greatest mortality in patients with SLE, as renal failure was the cause of death in one third of the patients. As to complications, secondary infections were recorded in almost 30% of the patients, incl. 9% lethal ones, non-infections complications less than 5%. The authors discuss assumed mechanism of the action of combined immunotherapy, which holds a priority position, as compared with glucocorticoid monotherapy.

In 15 patients with progressive systemic sclerosis with Raynaud's phenomenon Papaverin hydrochloride retard (Panergon) therapy was used. The average age of 14 females and 1 male was 42 years (26-55). Panergon therapy lasted for 4 weeks, the daily dose being 450 mg. For the evaluation of the treatment, 3 instrumental methods were used: IR radiometry for measuring thermal changes in the hands, capillaroscopy of nailfold, and Doppler-ultrasonic examination for measuring the circulation in finger arteries. An increased temperature, particularly in the fingertips, was found by radiometric examination in 6 patients. We also observed the acceleration of capillary flow speed in 7 patients. The pulse wave in finger arteries was markedly increased in 5 patients. From the subjective point of view the Panergon therapy was judged to be successful in 12 patients. It was not necessary to interrupt the treatment because of unfavourable effects in any case. Panergon therapy seems to be an important step in the treatment of Raynaud's phenomenon in progressive systemic sclerosis.