Osseous manifestations of sarcoidosis.

Sarcoidosis is a systemic multisystem inflammatory disease of unknown etiology. The disease is characterized by formation of non-caseating granulomas. The most common presentation is bilateral hilar lymphadenopathy and lung infiltration, but the disease is very heterogeneous, with an unpredictable clinical course. Musculoskeletal manifestations are common. Bone involvement is less frequent, and usually occurs in patients with chronic multisystem course of the disease. They are most commonly found in the phalanges of hands and feet, and are usually bilateral. The skull, long bones, ribs, pelvis, and axial skeleton may also be affected. Osseous involvement may be asymptomatic but in some cases can cause a severe disability. Imaging techniques are important for diagnosis. Radiological investigations revealed sclerotic or destructive lesions (involving also joints), cystic and punched out lesions and cortical abnormalities. Biopsy is required for differential diagnosis with respect to malignancy. Treatment is a part of systemic therapy and is not needed in all cases. Glucocorticoids and TNF-α antagonists are used for management.

Aggrecan Turnover in Women with Rheumatoid Arthritis Treated with TNF-α Inhibitors.

This study was performed to evaluate the effects of 15-month anti-tumor necrosis factor α (anti-TNF-α) therapy on the aggrecan turnover of female rheumatoid arthritis (RA) patients. Serum was obtained from healthy subjects and female RA patients treated with TNF-α inhibitors (TNFαI) in combination with methotrexate. We measured serum levels of aggrecan chondroitin sulfate 846 epitope (CS846), aggrecan fragments (AGC), disintegrin and metalloproteinase with thrombospondin motifs-4 (ADAMTS-4) and 5 (ADAMTS-5), as well as their natural inhibitor, known as tissue inhibitor of matrix metalloproteinase-3 (TIMP-3), using immunoassay methods. Serum levels of CS846, AGC, ADAMTS-4, ADAMTS-5 and TIMP-3 were higher in female patients with RA before the treatment in comparison to healthy subjects. Ratio of ADAMTS-5 to TIMP-3 was significantly higher in RA women than in controls, whereas ADAMTS-4/TIMP-3 ratio did not differ from that in controls. During the anti-TNF-α therapy, the serum levels of 846 epitope increased, whereas levels of AGC decreased in female RA patients. Furthermore, 15 months of treatment with TNFαI downregulated serum levels of both ADAMTS, without any effect on TIMP-3 levels. These changes were accompanied by significantly reduced ratios of ADAMTS to TIMP-3. According to our results, anti-TNF-α therapy has a beneficial impact on aggrecan remodeling during RA.

Effectiveness of subcutaneously administered methotrexate in patients with rheumatoid arthritis.

BACKGROUND: Subcutaneous methotrexate (sMTX) administration is considered more effective than the oral route due to better bioavailability and a lower rate of adverse drug reactions (ADRs); however, clinical data supporting this hypothesis is scarce. OBJECTIVES: The aim of the study was to evaluate the efficacy and tolerability of sMTX in patients with active rheumatoid arthritis (RA), including a subset classified as an early stage of RA. MATERIAL AND METHODS: A post-marketing, multicenter, open-label, non-randomized, non-interventional study enrolled 771 adult patients with active RA treated with sMTX (Metex®) for 2-6 weeks. The evaluation of therapy effectiveness (DAS28-ESR or DAS28-CRP) and monitoring of ADRs was an element of routine patient management. Therapy effectiveness was scored as the achievement of remission or response (according to European League Against Rheumatism (EULAR)). RESULTS: Among 761 (98.7%) patients that continued sMTX (after 25-31 weeks), clinical response was achieved by 69.5%, remission by 19.2% and low disease activity by 34.2%. Patients aged >60 years were less likely to achieve both remission (odds ratio (OR) = 0.61 (95% confidence interval (95% CI) = 0.39-0.93)) and clinical response (OR = 0.82 (95% CI = 0.71-0.95)), while overweight/obese patients (OR = 1.11 (95% CI = 1.00-1.24)) and those with early RA had greater chance to reach a clinical response (OR = 1.18 (95% CI = 1.03-1.34)). There were 16 ADRs (no serious or severe). In addition, at least 2-fold increase in alanine transaminase (ALT) activity was noted in 10 patients (1.3%). CONCLUSIONS: After 6-month therapy with sMTX, about 70% of patients with RA achieve a clinical response, and remission was observed in 20%. Younger age, overweight/obesity and an early stage of the disease are factors increasing therapy effectiveness; sMTX is well tolerated.

Effects of a 15-month anti-TNF-α treatment on plasma levels of glycosaminoglycans in women with rheumatoid arthritis.

BACKGROUND: In this study, the effect of 15-month anti-tumor necrosis factor alpha (TNF-α) treatment on circulating levels of plasma sulfated glycosaminoglycans (GAGs) and the nonsulfated GAG hyaluronic acid (HA) in female rheumatoid arthritis (RA) patients was assessed. METHODS: Plasma was obtained from healthy subjects and RA women treated with TNF-α antagonists (etanercept or adalimumab or certolizumab pegol) in combination with methotrexate. GAGs were isolated from plasma samples using ion exchange low-pressure liquid chromatography. Total sulfated GAGs were quantified using a hexuronic acid assay. Plasma levels of keratan sulfate (KS) and HA were measured using immunoassay kits. RESULTS: Total sulfated GAGs and HA levels were higher in female RA patients before treatment in comparison to healthy subjects. KS levels did not differ between RA women and controls. Anti-TNF-α treatment resulted in normalization of plasma total GAG and HA levels in RA patients, without any effect on KS levels. CONCLUSIONS: Our results suggest that anti-TNF-α therapy has a beneficial effect on extracellular matrix remodeling in the course of RA.

Coexistence of systemic sclerosis and sarcoidosis.

Coexistence of systemic sclerosis (SSc) and sarcoidosis (SA) is rarely reported; 21 cases only were reported in the English medical literature before 2011. It is suggested that low incidence of overlap syndrome of SSc with SA is resulted from different immune mechanisms involved in pathogenesis of the diseases. In SSc patients, a role of Th2 lymphocytes is suggested while in patients with SA such role is attributed to Th1 lymphocytes. The paper presents a 47-year-old woman suffering from SSc for over 6 years. CT scan of the lungs revealed the nodulus of the right lung and enlarged mediastinal lymphatic nodes. Pathologic evaluation of the nodulus provided basis for diagnosis of sarcoidosis. Diagnosis of SSc was based on clinical and capilaroscopic evaluation as well as detection of anti-topoisomerase I antibodies. In the course of the disease, fibrosis of the lung, pulmonary hypertension and cardiac abnormalities with rhythm disturbances were developed. Treatment included cyclophosphamide, mycophenolate mofetil, sildenafil, losartan. Stabilization of the general state of the patient was achieved.

Medical terminology: Its size and typology.

Medical terminology is one of the largest specialized terminologies and is estimated to contain over 250,000 items. Classification of medical terminology into six categories is proposed. The categories are as the following: (A) medical terms that are a part of general basic lexicon of average native speaker (0.02-0.03 % of all terms), (B) specialized medical terms known by average physician (about 45 % of all terms), (C) highly-specialized terms of subspecialties (about 15 % of all terms) (D) medical terms that primarily belong to other terminologies (e.g. biological, chemical, physical, statistical) (about 20 % of all terms), (E) medical slang (0.04-0.05 % of all terms), and (F) pharmaceutical terminology (about 20 % of all terms).

[The use of blood and its components in two gynecological-obstetrical wards of selected Silesian hospitals in years 1996-2002]. / Wykorzystanie krwi i jej skladników w dwóch oddzialach ginekologiczno-poloiniczych szpitali województwa slaskiego w latach 1996-2002.

UNLABELLED: Blood and its components are valuable medication that should be administered according to recommendation after consideration of expected benefits and adverse reactions to the patient. THE AIM OF STUDY: To analyze amount and form of blood components or whole blood transfused at gynecological-obstetrical wars of university hospital (UH) or regional hospital (RH). Age of the patients and clinical diagnosis were included into the analysis. MATERIAL AND METHODS: Two gynecological-obstetrical wards were investigated. The appropriate data were obtained from the hospital documentation (there was no electronic data system). Amount and form of transfused blood components or whole blood, diagnosis at admission, patient's age and number of the treated patients were collected from the hospital transfusion book. The final clinical diagnosis, age of the patient, and transfusion details were obtained from the patient's medical records. The diagnoses were presented according to the ICD-10 classification. UH patients (29,759 patients) and 13,540 patients from RH from 1996 to 2002 were investigated. RESULTS: Blood and its components were used for transfusion in 1150 women (3.8%) treated in UH, and in 206 women (1.5%) hospitalized in RH. In years 1996-2002, there were transfused 2746 units of blood and its components, including: 371 units of whole blood (13.5%), 2073 units of red blood cells (75.5%), 281 units of fresh frozen plasma (10.2%) and 21 units of therapeutic platelet concentrate (0.8%) in RH there were transfused 527 units of blood and its components, including: 8 units of whole blood (1.5%), 450 units of red blood cells (85.4%), 63 units of fresh frozen plasma (12%), and 6 units of therapeutic platelet concentrate (1.1%). The age of patients in UH was between 14 and 92 years and in RH between 18 and 79 years. CONCLUSIONS: The rate of patients with the same diagnosis in whom the transfusion was made, was higher in University Hospital. There were no differences between the number of transfused blood and its components between the two hospitals. The age of patients who had blood transfusion was higher for the University Hospital. Introduction of payment for blood and its components, resulted in decrease in percentage of the patients receiving transfusion as seen in comparison of the period 1999-2002 and 1996-1998 in both investigated hospital. The PRBC transfusion average rate units, increased in the period 1999-2002, compared to the 1996-1998. Presented method of analysis of use of blood and its components may reveal new aspects of the problem and may be helpful for planning of blood donation.

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.

[Differential diagnosis of fever]. / Diagnostyka róznicowa stanów goraczkowych.

Fever is a common clinical phenomenon. In most cases, the cause of fever can be determined quickly. The clinical problem starts when the patient has fever for a long period without any easily detectable cause. The cause of fever can be classified as follows: infectious agent, neoplasm, immune or metabolic disorder, genetic abnormality of inflammasome functioning. The clinical course of fever may vary (continuous, remittent, intermittent, hectic). Detection of the cause of chronic fever is not always possible. In these cases fever of unknown origin is diagnosed.

Alkaptonuria.

Alkaptonuria is a hereditary disease resulted from accumulation of homogentisic acid within the body due to deficiency of homogentisic acid oxidase. The main clinical feature is dark brown color of urine caused by high urinary output of homogentisic acid. There are no other symptoms or signs of the disease until the fourth decade of life when ochronosis is developed. Life-long accumulation of abnormal metabolites becomes overt in form of severe spondylosis, peripheral arthropathy, tendon rupture, bone osteoporosis as well as aortic valve stenosis and skin pigmentation. The features of the disease are associated with affinity of homogentisic acid to the connective tissue and its effect on collagen structure. Only symptomatic treatment is applied in case of alkaptonuria and ochronosis.

[Biological agents in treatment of psoriatic arthritis]. / Zastosowanie leków biologicznych w leczeniu tuszczycowego zapalenia stawów.

Psoriatic arthritis was reported in approximately 6-39% of patients with psoriasis. It is a chronic disease that commonly leads to disability. The paper reviews TNF-alpha inhibitors, the drugs which resulted in a substantial progress in management of psoriatic arthritis. Numerous studies have shown efficacy of these medical agents including inhibition of the disease progress, improvement of skin changes, inhibition of bone destruction, and improvement in quality of life. Other agents which can be used in psoriatic arthritis treatment are being tested in clinical trials.

[Wladyslaw Antoni Gluzinski--an eminent Polish internist, founder of the Polish Society of Internal Medicine]. / Wladyslaw Antoni Gluziniski--wybitny internista polski, zalozyciel Towarzystów Internistów Polskich.

Wladyslaw Antoni Gluzinski is one of the greatest physicians in history of Polish medicine. He was born on May 18, 1856 in Wloclawek. Studied medicine at the Jagiellonian University in Krakow (1874-1880). He was working in Krakow, Lvov and Warsaw. He died on April 10, 1935 in Warsaw. Wladyslaw Antoni Gluzinski was one of the pioneers of Polish gastroenterology. He introduced the functional test for detection of the stomach cancer (the Gluzinski's test), and was the author of the first clinical description of plasmocytic leukemia. He was a founder of the Polish Society of Internal Medicine and the first editor-in-chief of the Polish Archives of Internal Medicine as well as he initiated meetings of Slavic physicians.

[Michal Litynski--a forgotten author of the first description on primary hyperaldosteronism]. / Michal Litynski - zapomniany autor pierwszego opisu hiperaldosteronizmu pierwotnego.

Primary hyperaldosteronism (Conn's syndrome) was described for the first time by the Polish internist Michal Litynski (1906-1989) in 1953. Jerome W. Conn's (1907-1994) report on the disease appeared in 1955.

[The role of adiponectin in atherosclerosis]. / Rola adiponektyny w miazdzycy.

Adiponectin is a adipose tissue-derived protein. In the light of current investigations, the role of adiponectin goes far beyond only adipose tissue regulatory factor, being involved in some pathological processes as endothelium damage or atherosclerosis. Unique properties of adiponectin make it a very promising agent that posses ability to slow down the progression of atherosclerosis. In this article, a structure and a function of adiponectin have been reviewed. The special emphasis was put upon the role of adiponectin in atherosclerosis.