Hungarian Immunology

[Autoimmunity as a result of escape from RNA surveillance]


JANUARY 20, 2007

Hungarian Immunology - 2007;6(01-02)

[The pathomechanisms of autoimmune diseases are still unknown. Numerous factors are thought to play a role in the formation of the diseases (genetic arrangement, hormonal factors, exogen and endogen viruses, etc.) and many hypotheses have been formulated to explain the role of these factors. Most of the theories suspect that disturbance of the immune system is the clue but according to other researchers the immune system performs properly and one has to find other alterations that could be blamed for the formation of the autoimmune diseases. The aim of our present work is to show that certain genetic alterations together with the mistake of the RNA surveillance system could lead to autoimmune reactions. Results of immune research conducted in the past two decades revealed that there are mutations in the hot spot region of exon 7 of the La gene in the peripheral lymphocytes of patients suffering from certain autoimmune diseases (Sjögren's syndrome, SLE). RNAs originated from the mutant gene contain premature termination codon and therefore the RNA surveillance mechanism should get rid of these RNAs in order to prevent the formation of mutant proteins. However, because of the mistake of the surveillance system mutant proteins are formed that could finally lead to the autoimmune reactions.]



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[Biological therapy acts at a specific point of inflammation. Today, rheumatoid arthritis is the prototype disease in this context due to the relatively high number of accessible patients and wellstandardized follow-up tools. However, apart from this disease, biological therapy has been introduced to the treatment of other diseases including various forms of arthritis, such as ankylosing spondylitis and psoriatic arthritis, as well as systemic autoimmune disorders, such as lupus, scleroderma, inflammatory myopathies and Sjogren’s syndrome. Anti-tumor necrosis factor-α (TNF-α) agents play a central role in biological therapy as these agents have been successfully tried in most of these diseases. However, when seeking for specific targets for biologicals, specific pathogenic factors of the given disease should be determined. For example, while anti-TNF agents seem to be most effective in various types of arthritis, anti-B cell therapy may be the first choice in other autoimmune conditions, such as lupus, Sjogren’s syndrome or dermatomyositis. It is important to consider the specific characteristics of the administered agents as there may be differences regarding side-effects.]

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[INTRODUCTION - In some cases of polymyositis/ dermatomyositis (PM/DM) of autoimmune origin, different malignant tumours can initiate the difficult cascade mechanisms at cell level leading to the rapid weakness of the skeletal muscles. Till now, in the international literature only four cases of PM/DM associated with cancer of prostate has been reported. CASE REPORT - Authors present a case of a 57 yearold male patient, where weakness in patient's movements developed leading to total immobility in 3 months. Purple discoloration developed on his hand and face. Significantly elevated creatin kinase (CK) levels and blood sediment rate with mild anaemia were observed during laboratory examinations. Dysphagia and lack of appetite resulted in the loss 10 kgs in body weight. Both clinical evaluation, elevated serum CK level, skin symptomes, positive electromyography and muscle biopsy confirmed acute definitive dermatomyositis. Urological examination revealed a palpable hard area at the right lobe of the prostate. Prostate biopsy confirmed the presence of carcinoma in the right lobe of the prostate. There was no sign or symptome referring to either local propagation or distant manifestation. Therefore, radical prostatectomy was performed, the tumorous prostate and both seminal vesicles were removed. Histological examination proved malignant focus in the right lobe of the prostate. After the operation patient gradually became stronger, corticosteroid medication were decreased then stopped. Patient's original muscle power and movement recovered and his previous body weight was regained. During the regular control examinations all results of laboratory tests are in normal range. CONCLUSION - In the background of some autoimmune diseases malignant tumours can be revealed. It is rare when urological neoplasms initiative the process. In men with PM/DM commencing beyond 50 years of age it is necessary to think of the presence of prostate cancer, which can be cured by performing radical operation in appropriate time.]