Hungarian Immunology

[MCP-1 (monocyte chemoattractant protein-1) G/A and T-bet (T-helper promoter factor) C/G polymorphisms in primary Sjögren’s syndrome and systemic lupus erythematosus]

KOVÁCS Attila, KONCZ Ágnes, ENDREFFY Emőke, ARANKA László, PETRI Ildikó, ELLER József, SZALAI Csaba

JANUARY 22, 2008

Hungarian Immunology - 2008;7(01-02)

[INTRODUCTION - Monocyte chemoattractant protein- 1 (MCP-1) is a β-chemokine involved in the attraction and accumulation of mononuclear granulocytes towards the site of inflammation. One of the transcriptional factors of T-cells is called T-bet. PATIENTS AND METHODS - The authors investigated the MCP-1-2518 G/A and T-bet 310 C/G (His33Gln) polymorphisms evaluating the distribution of the specific genotypes in 45 patients with primary Sjögren's syndrome (pSS), 51 patients with systemic lupus erythematosus (SLE), and in 320 healthy blood donors as the control group. MCP-1-2518 G/A and T-bet 310 C/G polymorphisms were detected with molecular genetic methods from the purified genomic DNA. RESULTS - The frequency of the MCP-1-2518 AG heterozygous genotype decreased tendentiously only in SLE patients, while the frequency of the MCP-1 AA homozygous genotype increased comparing to the control group (13.7% vs. 5.9%; Pearson’s χ2 test=6.125, ns.). Analyzing the genotype frequency for the MCP-1 wild (GG) and AA homozygous genotypes in pSS group, the MCP-1 AA homozygous genotype proved to be more frequent comparing to the control group (82.8%:17.2% vs. 90.7%:9.3%; Pearson’s χ2 test 1.755, ns). These relations showed only tendentious association in the SLE group (81.6%:18.7% vs. 90.7%:9.3%; Pearson’s χ2 2.811, p=0.094, ns.) There was not any significant correlation between the investigated MCP-1- 2518 G/A and the T-bet 310 C/G polymorphisms and the TNF-α -308 G/A and -238 allele polymorphisms. The frequency of T-bet was equal in relation with heterozygous (CG) to wild CC genotype in the investigated two autoimmune disorders. The GG homozygous genotype for T-bet could not be found in SLE and pSS groups, likely to be a protective factor. CONCLUSIONS - The above mentioned polymorphisms didn’t show any significant correlation with TNF-α -308 and -238 allele polymorphisms. The further research of the MCP-1 G/A and T-bet C/G polymorphisms is important, because of their possible prognostic importance for SLE and pSS.]



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Hungarian Immunology

[Pseudolymphoma orbitae]

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[INTRODUCTION - Pseudolymphoma orbitae is a rare and difficult entity. The cooperation of the pathologist and clinician is needed to properly manage the patient. CASE REPORT - The authors report the case history of a 38 years old male patient. His disease started at the age of 30. He was previously treated with allergic rhinitis. No definitive diagnosis was made for eight years. Several surgical biopsies were made from nasal mucosa, but no specific histologyical diagnosis was applicable. At the age of 30 he developed an unilateral exophthalmus on the left side. Thyroid associated ophthalmopathy was ruled out several times with laboratory analysis. High dose methylprednisone therapy was repeatedly given with limited results. At the age of 34 orbital CT and MRI scan confirmed the pseudotumour orbitae already compressing the optical nerve. Laboratory analysis again ruled out thyroid associated ophthalmopathy. Churg-Strauss syndrome, Wegener’s granulomatosis or Sjögren’s syndrome could be ruled out. A bone marrow trephine biopsy excluded systemic hematological disease as well. A biopsy was performed from the retrobulbar mass again, which confirmed the lymphoid hyperplasia with B-cell dominance. High dose methylprednisone and local irradiation resulted only moderate decrease of the mass, so systemic chemotherapy was started using CVP (cyclophosphamide, vincristin, prednisone) then CHOP (CVP + anthrycycline) polychemotherapy for eight cycles and subcutaneous interferon-α for 20 months. CONCLUSIONS - This resulted a complete regression of the disease, and the patient is well for 48 months now.]

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[SS-A(Ro) and SS-B(La) autoantibodies in systemic lupus erythematosus]

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[OBJECTIVE - To assess the relation between clinical features and the presence of SS-A(Ro) and SS-B(La) autoantibodies in systemic lupus erythematosus. PATIENTS - The data of 200 patients with definite systemic lupus erythematosus were analysed. SSA( Ro) and SS-B(La) antibodies were assessed by enzyme immunoassay. RESULTS - 40.5% of systemic lupus erythematosus' patients were SS-A(Ro) and/or SS-B(La) antibody positive (’positive group’); the majority of such patients displayed both antibodies, 16.5% had SSA( Ro) antibodies alone, while only 2% has SS-B(La) antibodies alone. There were no differences in the occurrence of arthritis, secondary antiphospholipid syndrome and hematologic manifestations between the positive and negative groups; serositis was more common in the positive group. Skin manifestations, in particular subacute cutaneous lupus erythematosus and urticaria vasculitis were more frequent in the positive group, while kidney and central nervous system involvation, in particular severe forms were less frequent. Secondary Sjögren's syndrome occurred exclusively in antibody positive patients. Sm, RNP and Scl-70 antibodies were more frequently found in the positive group. CONCLUSIONS - The presence of SS-A(Ro) and/or SS-B(La) antibodies in systemic lupus erythematosus has some prognostic significance; in antibody-positive patients there is an increased risk for skin lesions (in particular subacute cutaneous lupus erythematosus and urticaria vasculitis) and secondary Sjögren’s syndrome and a decreased risk for severe nephritis or central nervous system involvement.]

Hungarian Immunology

[The outcome of the renal involvement in primary Sjögren’s syndrome]


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Lege Artis Medicinae



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Clinical Neuroscience


ILNICZKY Sándor, KAMONDI Anita, ARÁNYI Zsuzsanna, VÁRALLYAY György, GAAL Barbara, SZIRMAI Imre, NAGY György

[Systemic lupus erythematosus is a frequent autoimmune disease, affecting several organs, including the brain, spinal cord and nerves. Cerebral vasculitis, transverse myelitis and polyneuropathy are the most common neurological manifestations. We report a case of a 46 years old woman who suffered incomplete transverse myelitis in her age of 44. After 2 years the second relapse presented with arthralgias, painful paraesthesias and weakness of the lower limbs. Neurological signs suggested involvement of the central and the peripheral nervous system. Based upon clinical and laboratory findings systemic lupus erythematosus was diagnosed. Magnetic resonance imaging revealed two hyperintense lesions on T2 weighted scans within the cervical spinal cord. The brain scan was normal. Protein content was slightly elevated in the cerebrospinal fluid, with normal cell count. Electrophysiological examinations diagnosed a subacute sensory-motor axonal polyneuropathy. On methylprednisolone treatment her condition improved. Simultaneous development of central and peripheral lesions of the nervous system in cases with systemic lupus erythematosus may lead to a challenge to establish the diagnosis.]

Clinical Neuroscience

Posterior reversible encephalopathy syndrome as an initial manifestation of systemic lupus erythematosus

AYAS Özözen Zeynep, ÖCAL Öncel Ruhsen, GÜNDOGDU Aksoy Asli

Posterior reversible encephalopathy syndrome (PRES) is a disorder which is diagnosed with its characteristic clinical and radiological findings, typically resolves with treatment. The prevalence of PRES in systemic lupus erythematosus (SLE) patients is not exactly known. A systemic disorder frequently appears as a presenting symptom in SLE. However, in rare cases, the disease starts with a neurological manifestation. Here we report a 35-year-old woman presenting with a headache and blurred vision. She had neurologic symptoms and cerebral lesions on magnetic resonance imaging (MRI) suggesting PRES. The patient was diagnosed with SLE during the etiological investigation of PRES. In this article, we aimed to emphasize that PRES as an initial presentation of SLE.