Impact of systemic steroids combined with immunosuppressive treatment on glaucomatous features in patients with systemic lupus erythematosus.

AIM: To evaluate the incidence of increased intraocular pressure (IOP) and glaucomatous changes in systemic lupus erythematosus (SLE) patients in comparison with systemic steroids and immunosuppressive treatment. METHODS: Sixty-two women with SLE were divided into two groups: treated (n=47, 94 eyes) and not treated (n=15, 30 eyes) with systemic glucocorticosteroids (GC; GC-free). Twenty-one individuals in GC group were treated with immunosuppressive agents (immunomodulating and biologic). The visual acuity and IOP with ocular pulsatile amplitude (OPA) measurements, as well as scanning laser polarimetry (GDx) with nerve fiber index (NFI) measurement, spectral domain optical coherence tomography (SD-OCT) of the optic disk with retinal nerve fiber layer (RNFL) analysis and the macular region with ganglion cell analysis (GCA) were performed. RESULTS: Mean IOP values in group with combined GC and immunosuppressive therapy was 15.8±2.56 mm Hg and was significantly lower than in individuals with exclusive GC treatment (17.63±4.38 mm Hg, P=0.043). Contrary, no differences in mean IOP values between GC-free group and individuals treated with combined GC and immunosuppressive therapy were detected (P=0.563). Similarly, mean IOP in GC was 17.14±3.94 mm Hg and in GC-free patients was equal to 16.67±3.45 mm Hg (P=0.671). According to treatment regimen no statistical differences in optic disk SD-OCT for RNFL thickness, RNFL symmetry, cupping volume and the C/D ratio were observed. Similarly, no statistical differences for the mean and minimal ganglion cell layer (GCL) thickness measured in macular SD-OCT or NFI in GDx were detected. CONCLUSION: Combined immunosuppressive and systemic GC therapy in SLE patients may lower the risk of iatrogenic ocular hypertension. No relationship between treatment regimen and glaucomatous damage of optic nerve fibers in analyzed groups with SLE is detected.

PTPN22 1858C>T gene polymorphism in patients with SLE: association with serological and clinical results.

To assess the association between PTPN22 1858C>T gene polymorphism and susceptibility to, and clinical presentation of, systemic lupus erythematosus (SLE). Our study included 135 SLE patients (120 women and 15 men; mean age 45.1 years; mean course of disease from 0.5 to 31 years) and 201 healthy subjects. The PTPN22 1858C>T gene polymorphism was genotyped by polymerase chain reaction restriction fragment length polymorphism. A significantly higher incidence of genotype CT in patients with SLE (36.3 %) was found, compared with the control group (24.9 %). The frequencies of C1858 and T1858 alleles were 78.1 and 21.9 % in SLE patients and 86.1 and 13.9 % in controls, respectively. Significantly higher SLE susceptibility was observed in patients carrying at least one T allele (p = 0.009; OR 1.86; 95 % CI 0.14-3.05). Significant association of the PTPN22 T1858 allele (CT + TT vs.CC) and secondary antiphospholipid syndrome was observed (p = 0.049). In SLE patients carrying the T1858 allele, higher levels of antiphospholipid antibodies (anticardiolipin antibodies and/or lupus anticoagulant) were found (p = 0.030; OR 2.17; 95 % CI 1.07-4.44).

Polyarteritis nodosa and Sjögren's syndrome: overlap syndrome.

Polyarteritis nodosa (PAN) belongs to a group of necrotic angiitis. During the illness, necrotic changes are found in small and middle dimensions arteries. Primary Sjögren's syndrome is a chronic, autoimmunological systematic illness of connective tissue with characteristic infiltration of lymphocytes and plasmatic cells in endocrine glands. Despite the fact that both disease entities are well known and primary Sjögren's syndrome is the second most commonly appearing autoimmunological sickness, the coexistence of both simultaneously is described very rarely. So far only three such cases have been presented. The case of 53-year-old woman is presented, who since 2003 has been hospitalized due to her ailments several times, at surgery, internal medicine, and rheumatology wards. In 2006, she was admitted to rheumatology clinic of Pomeranian Medical University (PAM) to be diagnosed both subjectively and objectively. Additional examinations proved that she had been suffering from overlapping PAN and primary Sjögren's syndrome (PSS). She fulfilled 5 out of 10 criteria for PAN and all criteria for PSS. For treatment the boluses of methyloprednisolon and cyclophosphamid every 4 weeks were used what resulted in curing the patient.

[Analysis of the clinical picture and serologic profile in patients with dermatomyositis and polymyositis coexisting with neoplastic disease]. / Analiza obrazu klinicznego i profilu serologicznego u chorych na zapalenie skórno-miesniowe i zapalenie wielomiesniowe ze wspólistniejaca choroba nowotworowa.

INTRODUCTION: There is much evidence on associations of dermatomyositis (DM) and polymyositis (PM) with malignant neoplasms. The prevalence of malignancies in patients with DM/PM is estimated at 6 to 60%. This study was undertaken to analyze the clinical and serologic picture of DM and PM coexisting with neoplastic disease. MATERIAL AND METHODS: We studied 61 patients (37 with PM, 16 with DM, and 8 with the overlap syndrome - OVS). Diagnostic imaging, endoscopy, electromyography, histopathology of skin and muscle biopsies, echocardiography, and biochemical tests were done in all patients. The profile of antinuclear antibodies (ANA), myositis-specific autoantibodies (MSA), and myositis-associated autoantibodies was determined as well. Correlations of neoplastic disease with clinical, laboratory, and serologic parameters in patients with DM or PM were studied with logistic regression analysis. RESULTS: Neoplastic disease was diagnosed in eight patients (five with DM, two with PM, and one with OVS). Neoplastic disease was the cause of death in five patients. The mean time from diagnosis of DM/PM to diagnosis of tumor was 2.44 +/- 2.5 years. The most frequent clinical symptoms in patients with coexisting neoplastic disease were: dysphagia (8), myalgia (8), fever (7), skin lesions (5), pruritus (4), joint pain/effusion (4), and resistance to treatment (4). ANA were detected in six patients. None of the patients with a neoplasm tested MSA-positive; other antibodies were found in some patients. Risk factors for neoplastic disease included anemia at the time of diagnosis (OR: 16.75; 95% CI: 1.81-154.60; p = 0.013) and dysphagia (OR: 5.08; 95% CI: 1.23-243.90; p = 0.031). CONCLUSION: Dysphagia and anemia at onset of the disease in a patient with idiopathic inflammatory myopathy are symptoms which suggest the risk of a tumor.

Ocular circulation in systemic lupus erythematosus.

BACKGROUND: Mean vascular resistance in the retrobulbar arteries of SLE patients and the statistical relationship between its parameters and the presence of certain antibodies were determined. MATERIAL/METHODS: Forty-three eyes of 43 SLE female patients aged 46.28+/-8.45 years with disease duration of 10.03+/-7.96 years were examined. Physical and ophthalmic examinations with assessments of the immunological markers ANA/IgG-IgM, aCL, anti-beta2GPI, LA, and anti-dsDNA antibodies were performed. color Doppler imaging (CDI) was used in the OA, CRA, LPCA, and MPCA vessels. The vascular resistance indices (RIs) were compared with those of 43 eyes of 43 female controls. Covariance and multiple regression analysis with chi squared, Pearson, Shapiro-Wilk, and Levene tests were used in the statistical analysis (significance levels at p

[Eosinophilic fasciitis--diagnostic and therapeutic difficulties]. / Eozynofilowe zapalenie powiezi--trudnosci diagnostyczne i terapeutyczne.

Eosinophilic fasciitis is a rare disease classified by some authors to scleroderma like syndromes. It occurs the most frequently between the second and the sixth decade of life, mainly in Caucasians. It usually appears in young males and exceptionally in children. It has an abrupt onset. The etiology of this disease is unknown. Hardening of the skin and subcutaneous tissue, eosinophilia in peripheral blood and hypergammaglobulinaemia are the most characteristic features of the disease. Inflammation and fibrosis of the fascia that spreads over into the deeper layers of skin and muscles are typical for its histological picture. The infiltration is composed of the lymphocytes, plasmocytes and eosinophiles. The involvement of internal organs in the course of the disease is rare. The course of eosinophilic fasciitis can be different. Prognosis is generally good, however recurrences of the disease can happen. The treatment with nonsteroidal anti-inflammatory drugs and/or with glucocorticosteroids is not satisfactory in some cases. It is not uncommon that intense immunosuppressive treatment is required.

[Chorea as a rare symptom of systemic lupus erythematosus in the elderly]. / Plasawica jako pierwszy, rzadko wystepujacy objaw tocznia rumieniowatego ukladowego u osób w podeszlym wieku.

The opinion that systemic lupus erythematosus (SLE) is a young people disease translates into its rare diagnosing in subjects in their 6th decade of life and older. The variability of neuropsychiatric disorders that may be the first symptoms of the disease suggest the need for their in-depth diagnostics and treatment. Chorea is one of the rarest neuropsychiatric symptoms of SLE. It may be present in an early stage of the disease and it is one of the most common motor disorders in SLE. The presented case of diagnosing SLE in a 79-year-old male shows the importance of individual and thorough assessment of the clinical picture of every patient regardless of his/her age and gender. In the discussed case the clinical course of the disease denies common opinions about the picture of SLE in the elderly.

[Fasciitis eosinophilica: personal observations and a review of the literature]. / Eozynofilowe zapalenie powiezi: opis 5 przypadków i przeglad literatury.

Eosinophilic fasciitis is a rare disease that is classified by some authors to scleroderma-like syndromes. Symmetrical induration of skin and subcutaneous tissue associated by eosinophilia in peripheral blood are characteristic features of the disease. Internal organ involvement is uncommon. It is often difficult to diagnose eosinophilic fasciitis and its course may be variable. Glucocorticosteroids are most commonly used in the treatment but in many cases they are ineffective. Then other immunosuppressive therapy must be considered. Prognosis is rather favorable. The remission is not always achieved and sometimes flares of the disease are observed as evidenced by the described cases. It should be emphasized that a majority of our patients were females. In four out of five patients anti-thyreoglobulin antibodies and/or anti-thyroid peroxidase antibodies were present suggesting their involvement in the pathogenesis of eosinophilic fasciitis. Neither indicators of inflammation nor peripheral blood eosinophilia were pathognomonic. Results of glucocorticosteroid treatment were satisfactory in three patients, but two patients required combined immunosuppressive treatment.

[Ocular manifestations in patients with systemic lupus erythematosus and antiphospholipid syndrome]. / Zmiany oczne u chorych na toczen rumieniowaty ukladowy i zespól antyfosfolipidowy.

INTRODUCTION: Systemic lupus erythematosus (SLE) is a systemic disease of connective tissue with broad band of symptoms. It could be the reason for many organs and tissues impairment. Changes in eyes that occurr in SLE are not frequent, but can lead to severe impairment of sight including blindness. OBJECTIVES: The aim of the work was to asses the frequency of eye changes among patients with SLE and SLE with antiphospholipid syndrome (APS). Another aim was to asses the association between antiphospholipid antibodies and ocular lesions. PATIENTS AND METHODS: There were 75 patients enrolled with SLE, 26 of them ha APS. All of patients had a comprehensive ophthalmological and physical examination. Moreover biochemica analysis including lipid profile and glucose metabolism and serological markers of APS and SLE were performe RESULTS: Thirty-six patients complained of ophthalmologic disturbances (48%), with "dry eyes" being the most common symptom (20 patients). The reduced visual acuity was detected in 17 patients (22.6%). Conjunctivitis was found in 8 patients (10.67%), corneal involvement in 31 (41.3%), and sclera changes in 40 patients (53.3%). Changes in retina were found in 15 (20%) of patients, the most frequent were sub-retinal edema in the region of yellow spot. Changes in yellow spot were found in 8 patients; in 2 of them it was associated with dry degenerative changes, in 6 patients exudates with or without hemorrhages were found. Vascular changes including their lumen diameter were found in 33 patients (44%). In 4 patients there were changes in optical nerve disc. Schirmer's test was pathological in 43 patients (57.3%), but in only 4 patients Sjögren's syndrome was diagnosed. In the group of SLE patients intraocular pressure was significantly higher. The presence of anticardiolipin antibodies IgG class (aCL IgG) was associated with reduced visual acuity. The presence of lupus anticoagulant and anti-beta2, glycoprotein-I antibodies (anti-beta2GPI) was associated with conjunctive involvement. The presence of aCL IgM and anti-beta2GPI was associated with less frequent symptoms of eye dryness. CONCLUSIONS: We found the following significant factors of the occurrence of eye involvement in our series of SLE patients: high activity of disease (conjuctiva, iris, uvea, retina, spot, vessels and optical nerve disc involvement), late diagnosis of SLE (retinopathy and conjuctive involvement), arterial hypertension (reduced visual acuity, cornea involvement, vessels involvement), age (reduced visual acuity, cornea involvement, retinopathy), glucose metabolism disorders (changes in optical nerve disc) and presence of anti-double stranded DNA antibodies (retinopathy).

[Therapy resistance antiphosholipid syndrome in the course of systemic lupus erythematosus: case report]. / Oporny na leczenie zespól antyfosfolipidowy w przebiegu tocznia rumieniowatego ukladowego: opis przypadku.

Antiphospholipid syndrome (APS) is one of the most common reasons of thromboembolic complications in the course of connective tissue diseases. There is a strong relationship between presence of antiphospholipid antibodies and the risk of thrombosis. APS related thromboembolic complications range from superficial vein thrombosis up to rapidly developing, life threatening, multi-organ embolism. We present a case of a 21-year old female who presented with myocardial infarction as the first symptom of systemic lupus erythematosus and APS. Afterwards she was consequently treated with antithrombotic and anti-aggregation agents but it did not prevent her from reoccurrence of middle retinal vein. During the 10-year follow up she presented with reoccurring neurological symptoms i.e.: headache, memory deficits, concentration loss and impairment of the cognitive functions. She was found many times to be positive for IgG and IgM anticardiolipin antibodies, anti-beta2glicoprotein-I antibodies and lupus anticoagulant.

[Severe neurological and obstetrical complications in a patient with antiphospholipid syndrome]. / Ciezkie powiklania neurologiczne i poloinicze u chorej w przebiegu zespolu antyfosfolipidowego.

Antiphospholipd syndrome (APS) is a disease characterised by venous and arterial thrombosis or recurrent foetal loss, which are associated with antiphospholipid antibodies or/and lupus anticoagulant. Clinical symptoms ofAPS are assocciated with presence of noninflammatory thrombosis ocluding arteries or venes. Symptoms of APS are often very dramatic what can be illustrated by the presented case of 40 year patient. At the age of 25 after a miscarriage the patient developed tetraparesis and motor aphasia in the course of thrombosis in central nervous system vessels. In physical examination besides neurological symptoms reticular livedo was found on the trunk and limbs. Serological tests have revealed presence of high titre of IgG and IgM anticardiolipin antibodies and lupus anticoagulant (LA). CT examination revealed hypodensic foci in left parietal lobe, and abnormal EEG findings were observed in fronto-temporal leads of left hemisphera of brain. At that point the patient did not meet the criteria of connective tissue diseases, including lupus. The diagnosis of primary APS was suggested. The patient received anti-aggregation treatment and also immunosuppressive drugs (azathioprine and prednison) due to progression of neurological manifestations. 3 years later, the second pregnancy ended in the 27th week with intrauterine fetal death. During the 3rd pregnancy, 2 years afterwards, the patient was treated with heparin, aspirin and intravenous immunoglobulin. The pregnancy finished with a successful delivery at term, the newborn was in good condition. During the following pregnancy the symptoms of preeclampsia occurred at 36/37th week but the newborn was delivered in a good condition after a caesarean section. At the age of 36 patient developed ischemic brain stroke with left-side hemiparesis inspite of anti-aggregation and immunosupressive (prednison and azathioprine) treatment. At that time homogenic type antinuclear antibodies (ANA) 1:2560, anti-beta2-glycoprotein antibodies (beta2-GPI), aCL antibodies in medium titer and thrombocytopenia were found. The patient was treated with heparin, cyclophosphamide and methyloprednisolon intravenously. During rehabilitation process gradual improvement of cognitive functions, speech and motorical functions was observed. Recently high titre of ANA and recurent thrombocytopenia < 100,000/mm3 were present. Maybe during the further follow-up, 14 years after the first symptoms of APS, the patient will develop full blown symptoms of SLE.

[Coexistence of hypothyroidism with polymyositis or dermatomyositis]. / Wspólistnienie niedoczynnosci tarczycy i zapalenia wielomiesniowego lub skórno-miesniowego.

INTRODUCTION: Muscle weakness with elevation of muscle enzymes may be a predominant manifestation of many diseases, among them myositis and hypothyroidism. MATERIAL AND METHODS: Between 1997 and 2005, polymyositis (PM) or dermatomyositis (DM) was diagnosed in 28 patients (7 males, 21 females) referred to our Department of Rheumatology. Coexistence of hypothyroidism and poly/dermatomyositis was established in seven patients (25%), all of them women. Five of them fulfilled the diagnostic criteria for PM, two for DM. The mean age at the time of diagnosis was 46.1 years (40-54 years). Four patients were diagnosed with chronic autoimmune thyroiditis, in two patients the autoimmune etiology was probable, whereas one patient was diagnosed with hypothyroidism secondary to strumectomy for follicular adenoma. We present and compare signs and symptoms characteristic for hypothyroidism and poly/dermatomyositis. Hypothyroidism may be masked by symptoms of poly/ dermatomyositis especially when the course of myositis is rapid. RESULTS: Every patient suspected of poly/dermatomyositis should be tested for thyroid hormone levels to exclude hypothyroidism with muscle weakness arising from the polymyositis-like syndrome or alternatively to confirm the coexistence of hypothyroidism and poly/dermatomyositis.

[Spastic tetraparesis and heart thrombus in a male patient as first symptoms of the antiphospholipid syndrome]. / Tetrapareza spastyczna i skrzeplina wewnatrzsercowa u mezczyzny jako pierwsze objawy zespolu antyfosfolipidowego.

PURPOSE: The antiphospholipid syndrome (APS) manifests itself with circulating anticardiolipid antibodies (aCL) and/or lupus anticoagulant (LA) associated with thrombi and emboli or with recurrent complications of pregnancy. MATERIAL AND METHODS: We present a case of a 50-year-old man who was diagnosed with the antiphospholipid syndrome on the basis of serologic findings and results of diagnostic imaging. Neurological symptoms in the form of memory deficits, disorders of orientation, and pyramidal-type bilateral hemiparesis more evident on the left side were reported by the patient over a period of two years. Magnetic resonance imaging (MRI) of the head revealed numerous vascular foci located in the cortical-subcortical areas of the brain. Ultrasound (USG) disclosed a large, irregular thrombus adhering to the margins of the mitral valve with signs of inflammation. aCL (high titers in both classes), LA, and anti-beta2-glycoprotein I antibodies (a-beta2GPI) were found in serum. a-beta2GPI and anti-nDNA antibodies were disclosed in the cerebrospinal fluid. RESULTS: Combined anticoagulant and anti-aggregation therapy was unsuccessful. It was then decided to use immunosuppression with intravenous cyclophosphamide and methylprednisone pulses every 4 weeks (7 cycles). USG at follow-up showed marked regression of the mitral thrombus with swollen margins of the mitral valve. MRI of the brain confirmed progression of the lesions described previously. CONCLUSION: The diagnosis of secondary antiphospholipid syndrome associated most probably with systemic lupus erythematosus (SLE) was made. It should be remembered, however, that diagnostic criteria for APL and SLE partially overlap.