Health care in patients with primary Sjogren's syndrome.

The aim of the present study was to define more accurately the necessary health care in patients with the primary Sjogren's syndrome and to compare it to those necessary for patients with another autoimmune disease--rheumatoid arthritis without a concomitant Sjogren's syndrome (Tab. 1, Ref. 5). Full Text (Free, PDF) www.bmj.sk.

Pulmonary function in patients with Sjögrens syndrome.

The primary Sjögren's syndrome is a chronic inflamatory autoimmune disease of the exocrine glands with various glandular and extraglandular (systemic) manifestations. Changes in exocrine function of glands--including bronchial, should influence the frequency of pulmonary affecting, hence functional disorders as well. Examining some standard parameters of pulmonary function can often give valuable information about pulmonary alterations. The lack of tipical alterations in pulmonary function in patients with primary Sjögren's syndrome, found by us, gives good reason to express the supposition that pulmonary engagement with this disease is insignificant in the majority of cases. (Tab. 1, Ref. 7.).

HLA system and autoantibodies in primary Sjögren's syndrome.

The primary Sjögren's syndrome is an autoimmune exocrinopathy in which is established a correlation between the production of antibodies and certain HLA determinants. A significant number of antigens from A, B, C, DR and DQ loci was investigated in 50 patients with primary Sjögren's syndrome with positive autoantibodies against dsDNA, ssDNA, Ro (SS-A), La (SS-B), RNP antigens, as well as with higher titer of anticardiolipin antibodies from IgM and IgG classes. The HLA antigens were investigated by a microlimphocytotoxic test and a prolonged test, while the autoantibodies by an indirect immunofluorescency, counter electrophoresis and ELISA. A significant correlation was established between HLA-A1 and HLA-DR3 and the anti-Ro, anti-La, anti-DNA and anti-RNP antibodies, as well as between the higher titer of JgM and JgG anticardiolipins and the HLA-A1, B8 and DR3 antigens. No association was found between the specific for the disease anti-Ro and anti-La autoantibodies and certain genetic markers. The achieved results confirm the hypothesis that the autoantibodies' production in primary Sjören's syndrome is to a certain extent genetically determined. (Tab. 1, Ref. 5.)

Primary Sjögren's syndrome complicated with development of non-Hodgkin's lymphoma - a case report and literature review.

Primary Sjögren's syndrome (PSS) is an autoimmune disease of the exocrine glands that is presented with progressive ocular and oral dryness, parotid enlargement and often with systemic (extraglandular) manifestations. In patients with PSS the risk of development of non-Hodgkin's lymphoma (NHL) is 44-fold compared to healthy population. The risk is associated with certain characteristics of the disease's course: recurrent changes in the parotid glands, lymph node enlargement, skin vasculitis, peripheral neuropathy, anemia and lymphopenia. According to the morphologic and phenotypic characteristics, B-cell low-grade lymphomas prevail. This communication presents a 65-year-old woman with PSS who developed a follicular type B-cell NHL 21 years after the autoimmune disease had been diagnosed. The analysis of our case and the literature review summarize the characteristics of the course of PSS, histologic variants, and evolution and prognosis of NHL in this kind of patients.

Locus DR in primary Sjögren's syndrome.

The primary Sjögren's syndrome (pSS) is an autoimmune exocrinopathy which is presenting with progressive dryness of the eyes and the mouth, changes in the parotid glands and not rarely with systemic (extraglandular) manifestations. The development of the immunogenetics proved that the course of the disease is genetically determined. HLA-antigens, associated with pSS differ in the separate races, populations and ethnic groups.

Wrist tuberculosis in cladribine--induced remission of hairy cell leukosis.

A 45-year-old man with "hand-shoulder" syndrome developing eight-months after Cladribine-induced remission of an 11-year-old hairy cell leukosis is presented. Wrist bone biopsy was performed because of failure of the algodystrophy treatment and radiographic findings of progressive osteoporosis. Caseating epiteloid granulomas abundant in Langhans cells were found histologically and later Mycobacterium tuberculosis species was isolated in culture specimen. Fistulas were formed that healed after a prolonged anti-tuberculosis therapy. The role of cellular immunity deficiency in Cladribine-treated hairy cell leukosis that predisposes to mycobacterial infection is discussed.