Satisfaction and discontent of Polish patients with biological therapy of rheumatic diseases: results of a multi-center questionnaire study.

OBJECTIVES: Biologics are medications widely applied in the management of inflammatory rheumatic diseases. The drugs were found to be effective but their application is associated with some disadvantages. Medication with biologics is relatively expensive, and in Poland, it is carried out in specialized centers. The study was designed to evaluate various aspects of satisfaction and dissatisfaction of Polish patients treated with biologics. MATERIAL AND METHODS: An anonymous questionnaire was distributed in 23 Polish rheumatological centers involved in the treatment; 1212 returned questionnaires were used for analysis. Responses were received from 606 patients with rheumatoid arthritis, 427 with ankylosing spondylitis, 117 psoriatic arthritis, and 62 adult patients with juvenile idiopathic arthritis (in whom administration of the drugs had been introduced before they were 18 years old). The investigated group constituted about one-fifth of all rheumatic patients on biologics in Poland. RESULTS: A beneficial or very beneficial influence of the medication on the state of physical health was found mostly in patients with rheumatoid arthritis (51.3 and 30.5%) and ankylosing spondylitis (51.0 and 36.8%). Family life was improved by the treatment especially in patients with ankylosing spondylitis (40.7 and 35.6% beneficial and very beneficial, respectively), sleep quality and sexual life mostly in those with ankylosing spondylitis (beneficial/very beneficial influence 41.5/38.4, and 38.7/23.9, respectively). There was a rather small influence of biological treatment on the financial situation of the patients. In general, satisfaction with the treatment was evaluated as positive or very positive in 88% of all investigated patients.In a significant part of the patients, transportation to the medical center was considered as a disadvantage of the treatment. About one-third of the patients considered laboratory and imaging tests to be done before initiation of the medication as a difficulty, and for about 40% waiting time for qualification for the medication was a significant disadvantage. The route of drug administration was without importance for 4/5 of the patients. CONCLUSIONS: Summing up, the results were similar in the patients suffering from various diseases although those with psoriatic arthritis felt the highest satisfaction (possibly due to the positive aesthetic effect), and those with ankylosing spondylitis had significant improvement in sexual life (probably due to younger age). Relatively low satisfaction was found in patients with juvenile idiopathic arthritis. There was a small influence of medication on financial status of the patients. Application of biologics has few disadvantages and most of them are associated with the organization of health services (waiting time for the tests, transportation to the medical centers).

Mixed generalized/juxta-articular form of Dercum's disease.

Dercum's disease is a rare disorder with subcutaneous formation of fatty tissue (lipomas) with symptoms of pain, fatigue, stiffness, weakness and in some cases arthritis. The rarity of this disease causes that it is not taken into consideration in the rheumatological differential diagnosis, so this short report draws attention to this rare disease.

Relationship between adiponectin, leptin, IGF-1 and total lipid peroxides plasma concentrations in patients with systemic sclerosis: possible role in disease development.

AIM: The relationship between adiponectin, leptin, insulin-like growth factor-1 (IGF-1) and total lipid peroxide (TLP) concentrations, and its possible role in the development of diffuse cutaneous systemic sclerosis (dcSSc), were evaluated in this study. METHODS, RESULTS: Plasma adipokines and IGF-1 levels were determined using the enzyme-linked immunosorbent assay method, whereas TLP levels were determined using a photometric test, in 36 dcSSc patients and 40 healthy controls matched by age, sex and body mass index (BMI). Plasma levels of adipokines were significantly lowered, while TLP and IGF-1 were increased in dcSSc patients compared to controls. Adiponectin correlated significantly with leptin (r = 0.44), TLP (r = -0.54), CRP (r = -0.47), erythrocyte sedimentation rate (ESR) (r = -0.40) and duration of disease (r = -0.44). A significant relationship was found between leptinemia and IGF-1 (r = -0.40), TLP (r = 0.44), duration of disease (r = -0.38) and BMI (r = 0.65). TLP correlated with IGF-1 (r = -0.43), C-reactive protein (r = 0.47), ESR (r = 0.49) and duration of disease (r = 0.46), while IGF-1 correlated with ESR (r = -0.40). CONCLUSIONS: Adipose tissue may play a complex role in the development of dcSSc, affecting both the metabolic state of the organism, as well as free radical-induced connective tissue degradation. Although, leptin seems to exert a pro-oxidative effect and both adiponectin and IGF-1 appear to prevent free radical damage, confirmation of the above effects requires further research.

Correlation between erythrocyte sedimentation rate and C-reactive protein level in patients with rheumatic diseases.

OBJECTIVES: Erythrocyte sedimentation rate (ESR) and serum level of C-reactive protein (CRP) are the acute phase reactants most commonly determined in patients with rheumatic diseases. The indices are affected by different factors, but both of them are applied for evaluation of the disease activity in patients with inflammatory disorders of the musculoskeletal system. MATERIAL AND METHODS: The authors compared the results of ESR and CRP, which were carried out during routine diagnosis in 200 patients admitted to the Department of Rheumatology. RESULTS: A significant correlation between ESR and CRP was found (ESR after 1 h/CRP: correlation coefficient 0.6944, ESR after 2 h/CRP: correlation coefficient 0.6126). There was no difference in ESR or CRP between male and female patients, and patients older than 40 years had higher ESR and CRP. CONCLUSIONS: The obtained results support the usefulness of both indices in the clinical practice of rheumatologists.

Alterations of plasma glycosaminoglycan profile in patients with rheumatoid arthritis in relation to disease activity.

BACKGROUND: Qualitative and quantitative evaluation of plasma glycosaminoglycans (GAGs) of rheumatoid arthritis (RA) patients in relation to disease activity estimated by DAS28 score was evaluated. METHODS: GAGs were quantified by hexuronic acid assay and electrophoretic fractionation. Keratan sulfate (KS) and hyaluronic acid (HA) were measured by immunoassay. RESULTS: Chondroitin/dermatan sulfate (CS/DS) and heparan sulfate/heparin (HS/H) in plasma of healthy subjects and RA patients were stated. Total GAGs, CS, HS/H and HA levels were higher in patients with high and moderate disease activity than in controls. Total GAGs and CS levels in patients with high disease activity were elevated in comparison to patients with low disease activity. HS/H levels in patients with high and moderate activity were elevated in comparison to those with low disease activity. KS levels were increased in all patient groups in comparison to controls. Total GAGs, CS, HS/H and HA levels were positively correlated with DAS28 and CRP. CONCLUSIONS: Structural tissue damage/remodeling of the extracellular matrix occurs in RA, which is reflected in the qualitative and quantitative changes of plasma GAGs. The above changes depend on DAS28 and may contribute to systemic changes in the properties of the extracellular matrix.

Opinion of patients with ankylosing spondylitis on risk factors impairing their quality of life.

We studied 54 patients with ankylosing spondylitis with questionnaire in order to determine their view on threat to quality of their life related to the disease. We have show that pain and significant disability are the main threats associated with the disease in view of the patients. Social aspects (losing of job or decreasing of income) are also important for the patients, while management of the disease is not considered as arduous. The results of patients' opinion may be helpful in designing of educational programs for them.

Serum ghrelin in female patients with rheumatoid arthritis during treatment with infliximab.

Ghrelin is a gastric hormone that posses multiple functions, including induction of growth hormone release, regulation of proinflammatory cytokines and control of food intake and energy homeostasis. A few reports on serum ghrelin level in chronic inflammatory states revealed contradictory results. The study was undertaken to determine ghrelin in patients with rheumatoid arthritis receiving infliximab, a TNF-α blocking agent. Serum ghrelin was determined in 18 female rheumatoid patients before the treatment with infliximab, 1 week after the first infusion and after 53 weeks of medication and compared with 15 age-matched healthy women. Serum ghrelin level was shown to be increased in the patients. A decrease in serum ghrelin level was found after the first infusion of infliximab and similarly decreased ghrelin level but still higher than in the control was shown in the 53rd week of medication. The obtained results suggest that ghrelin level is related to inflammation, and its serum level in patients with severe rheumatoid arthritis behaves similarly to acute-phase reactants.

Plasma leptin and neuropeptide Y concentrations in patients with rheumatoid arthritis treated with infliximab, a TNF-α antagonist.

TNF-α is one of the key proinflammatory cytokines in pathogenesis of rheumatoid arthritis (RA). TNF-α was also found to enhance synthesis of leptin. Leptin is mainly adipocyte-derived hormone controlling appetite and energy expenditure. It acts through inhibition of neuropeptide Y secretion. It is possible that TNF-α-induced leptin secretion contributes to body mass reduction in patients with RA. The study was designed to determine the influence of inactivation of the TNF-α with infliximab on plasma leptin and neuropeptide Y concentrations in patients with RA. Sixteen female patients with RA treated with infliximab and 16 healthy women were investigated. Plasma leptin and neuropeptide Y concentrations were determined before, during and after 1 year management of the patients with infliximab and were compared with body mass index and body fatty and lean mass. There was no difference in plasma leptin concentration between the rheumatoid patients before therapy and the controls (15.6 ± 1.85 and 14.5 ± 2.15 ng/ml, respectively). Neuropeptide Y concentration was higher in the patients than in the controls (54.5 ± 3.96 and 24.8 ± 2.80 pmol/l, respectively). Treatment with infliximab resulted in enhancement in leptin concentration (18.5 ± 2.34 ng/ml) and a slight increase in neuropeptide Y concentration (58.7 ± 4.66 pmol/l). Physiological relationship between leptin and body mass was shown in the patients and was not altered during the treatment. There was no significant correlation between the disease activity and plasma leptin or neuropeptide Y concentrations.

[The clinical spectrum of connective tissue diseases - a single intensive care experience]. / Analiza przypadków ukladowych chorób tkanki lacznej leczonych w oddziale anestezjologii i intensywnej terapii.

BACKGROUND: Systemic connective tissue diseases are relatively rare disorders of unknown origin, possibly related to autoimmunity. In the retrospective study, we analysed the records of nine patients suffering from connective tissue disorders who were treated during a two-year period in an intensive therapy unit. METHODS: Hospital medical records, observational charts and all other available medical documents were analysed. RESULTS: The clinical courses of 9 patients were studied. They included 4 with scleroderma, 3 with systemic lupus erythematosus, and 2 with inflammatory myopathy. They were admitted to the ITU because of sepsis and/or pneumonia, pulmonary haemorrhage, or cardiovascular failure. Three patients (2 with systemic sclerosis, 1 with systemic lupus erythematosus) died. CONCLUSIONS: A multidisciplinary approach to treatment is a key factor in the successful management of patients with rare diseases, such as connective tissue pathologies. Frequent infections with respiratory and/or circulatory complications are the most common causes of severe morbidity in these patients.

Exacerbated course of atrophoderma of Pasini and Pierini in patient with papillary cancer of the thyroid gland.

Atrophoderma of Pasini and Pierini is a benign cutaneous atrophy skin to morphea en plaques on repression and is considered as systemic sclerosis-like disorder. A case of female patient with atrophoderma of Pasini and Pierini in whom rapid eruption of new plaques was found after many years of stable disease in described. Papillary cancer of the thyroid gland was diagnosed and acceleration of atrophoderma should be considered as paraneoplastic syndrome.

Serum human cartilage glycoprotein-39 in patients with systemic sclerosis: relationship to skin and articular manifestation.

Human cartilage glycoprotein-39 (HC gp-30) is a secretory protein of several types of cells including chondrocytes. It has been suggested to be a laboratory index of joint damage. Thirty-two patients with systemic sclerosis (SSc) and 22 age-matched controls were investigated. An increased serum HC gp-39 level was shown in SSc patients and was found to correlate with inflammatory indices. There was no correlation with modified Rodnan score, joint involvement, or duration of symptoms of SSc. The obtained results indicate for possible relationship of HCgp-39 to inflammation but do not suggest determination of HC gp-39 as clinically applicable index of articular involvement in SSc patients.

Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database.

OBJECTIVES: To determine the causes and predictors of mortality in systemic sclerosis (SSc). METHODS: Patients with SSc (n=5860) fulfilling the American College of Rheumatology criteria and prospectively followed in the EULAR Scleroderma Trials and Research (EUSTAR) cohort were analysed. EUSTAR centres completed a structured questionnaire on cause of death and comorbidities. Kaplan-Meier and Cox proportional hazards models were used to analyse survival in SSc subgroups and to identify predictors of mortality. RESULTS: Questionnaires were obtained on 234 of 284 fatalities. 55% of deaths were attributed directly to SSc and 41% to non-SSc causes; in 4% the cause of death was not assigned. Of the SSc-related deaths, 35% were attributed to pulmonary fibrosis, 26% to pulmonary arterial hypertension (PAH) and 26% to cardiac causes (mainly heart failure and arrhythmias). Among the non-SSc-related causes, infections (33%) and malignancies (31%) were followed by cardiovascular causes (29%). Of the non-SSc-related fatalities, 25% died of causes in which SSc-related complications may have participated (pneumonia, sepsis and gastrointestinal haemorrhage). Independent risk factors for mortality and their HR were: proteinuria (HR 3.34), the presence of PAH based on echocardiography (HR 2.02), pulmonary restriction (forced vital capacity below 80% of normal, HR 1.64), dyspnoea above New York Heart Association class II (HR 1.61), diffusing capacity of the lung (HR 1.20 per 10% decrease), patient age at onset of Raynaud's phenomenon (HR 1.30 per 10 years) and the modified Rodnan skin score (HR 1.20 per 10 score points). CONCLUSION: Disease-related causes, in particular pulmonary fibrosis, PAH and cardiac causes, accounted for the majority of deaths in SSc.

Serum human cartilage glycoprotein-39 levels in patients with systemic lupus erythematosus.

INTRODUCTION: Human cartilage glycoprotein-39 (HC gp-39) is a protein secreted by various cells including chondrocytes. Serum HC gp-39 has been suggested to be a marker of cartilage damage. However, inflammation involving other sites than the joints is an additional factor that increases the serum level of HC gp-39. OBJECTIVES: The aim of the study was to evaluate the usefulness of HC gp-39 determination in serum of patients with systemic lupus erythematosus (SLE) as a marker of joint involvement. PATIENTS AND METHODS: Serum HC gp-39 levels were measured in 25 patients with SLE and 22 healthy controls. SLE activity was assessed by the Systemic Lupus Erythematosus Disease Activity Index, and articular involvement by calculating the number of swollen and tender joints. The markers of inflammation (erythrocyte sedimentation rate, C-reactive protein) were determined. RESULTS: We observed an increase in HC gp-39 in SLE patients. However, there was no correlation of this parameter with disease activity, inflammatory markers (except serum gamma globulin levels), and articular involvement. CONCLUSIONS: The study suggests that increased HC gp-39 in SLE patients results mainly from inflammation and is not useful as a marker of joint involvement.

Seronegative arthritis in patient with solitary bone plasmacytoma.

We report a patient with localized focus of the bone destruction due to a rare disease, solitary bone plasmacytoma (SBP). The patient suffered from arthritis, mimicking seronegative rheumatoid arthritis. To our knowledge, it is the first description of coexistence of arthritis and SBP.