Leczenie rytuksymabem ziarniniakowatości z zapaleniem naczyń i mikroskopowego zapalenia naczyń

  Od dawna poszukuje się nowych możliwości leczenia chorych z zapaleniami naczyń. Cyklofosfamid (CYC) i glukokortykosteroidy (GKS) są powszechnie stosowanymi lekami w leczeniu zapaleń naczyń. Powyższy sposób leczenia powikłany jest wieloma działaniami niepożądanymi CYC (niepłodność, uszkodzenie szpiku, krwotoczne zapalenie pęcherza moczowego oraz nowotwory), a także działaniami niepożądanymi po stosowaniu GKS. Poza za tym nie u wszystkich chorych uzyskuje się remisję, a ponadto częste są nawroty choroby. Poszukiwanie nowych rozwiązań w leczeniu tych chorób doprowadziło do zastosowania leków biologicznych. Wynikało to między innymi ze zwrócenia większej uwagi na redukcję toksyczności leczenia, częstości i ciężkości działań niepożądanych oraz z faktu, że około 20% chorych nie odpowiada na standardową terapię i utrzymywania się nadal wysokiego odsetka (50%) nawrotów choroby. Jednym z leków biologicznych, który okazał się skuteczny w leczeniu GPA i MPA jest rytuksymab (RTX). Rytuksymab jest skuteczny w leczeniu ciężkiej, aktywnej postaci zapalenia naczyń związanej z przeciwciałami ANCA. Odpowiedź na leczenie utrzymuje się długo i trwa dłużej u chorych wykazujących całkowitą odpowiedź kliniczną. Nie należy zapominać także o działaniach niepożądanych dobrze znanym reumatologom. Aktualnie wydaje się, że nie ma dobrze udokumentowanego badania, które leczenie jest lepsze RTX czy CYC z następnym leczeniem AZA lub MTX. Długo czekali chorzy i lekarze reumatolodzy na zatwierdzenie decyzji wprowadzenia w Polsce RTX do leczenia ziarniniakowatości z zapaleniem naczyń. Lek ten jest szansą na uzyskanie długiej remisji choroby u tych wszystkich chorych, gdzie mało skuteczny był cyklofosfamid. Forum Reumatol. 2016, tom 2, nr 1, 7–13 Słowa kluczowe: limfocyty B, rytuksymab, ziarniniakowatość z zapaleniem naczyń; mikroskopowe zapalenie naczyń

GIANT CELL ARTERITIS IN POLAND. HOW TO INCREASE DIAGNOSTIC RATE?

Background: Th e incidence rate of giant cell arteritis (GCA) in Department of Rheumatology, Internal Medicine and Geriatrics was raised 10 folds between 2000 and 2016. Objectives: In this presentation a practical question: ”how to increase GCA diagnostic rate?” was addressed, and illustrated with some clinical cases. Methods: GCA referrals to Department of Rheumatology in Szczecin were analyzed. Additionally GCA epidemiology in Poland was analyzed based on national insurance registry and compared with distribution of fast track GCA clinics. Results: Increase of diagnostic rate was due to mainly 2 factors: 1. Development of eff ective temporal / large vessels visualization techniques and introduction of fast track GCA diagnostics. 2. Better cooperation with ophthalmologist and other specialists (Table 1.) Visualization techniques introduced were temporal/large arteries Doppler ultrasound (fast track) sometimes supported by large arteries computed tomography, occasionally by temporal artery biopsy and marginally by PET-CT. Based on national insurance registry, that we’ve analyzed, GCA diagnostic rate in Poland improved in recent years. Th is seems to correlate with increased interest in GCA in some sites that organized fast track GCA clinics. Conclusions: Increase of GCA diagnostic rate requires further efforts. References: 1. Atlas of ultrasound application in large vessel arteritis: giant cell arteritis and Takayasu arteritis., Milchert M, Diamantopoulos A, Brzosko M; Wydawnictwo Pomorskiej Akademii Medycznej, Szczecin, 201

Antiphospholipid Syndrome with Antiβ2glicoprotein-1 Antibodies as the Cause for Recurrent Tibial Vein Thrombosis in SAPHO Syndrome A case report

No abstract available</p

The expansion of CD4(+)CD28(- )T cells in patients with rheumatoid arthritis

Clonal expansion of CD4(+)CD28(- )T cells is a characteristic finding in patients with rheumatoid arthritis (RA). Expanded CD4(+ )clonotypes are present in the peripheral blood, infiltrate into the joints, and persist for years. CD4(+)CD28(- )T cells are oligoclonal lymphocytes that are rare in healthy individuals but are found in high percentages in patients with chronic inflammatory diseases. The size of the peripheral blood CD4(+)CD28(- )T-cell compartment was determined in 42 patients with RA and 24 healthy subjects by two-color FACS analysis. The frequency of CD4(+)CD28(- )T cells was significantly higher in RA patients than in healthy subjects. Additionally, the number of these cells was significantly higher in patients with extra-articular manifestations and advanced joint destruction than in patients with limited joint manifestations. The results suggest that the frequency of CD4(+)CD28(- )T cells may be a marker correlating with extra-articular manifestations and joint involvement

Leczenie łuszczycowego zapalenia stawów biologicznymi lekami modyfikującymi

Skuteczność tradycyjnych leków modyfikujących przebieg choroby w hamowaniu uszkodzenia strukturalnego stawów w ŁZS jest ograniczona, a ich hepatotoksyczność częstszą niż w innych chorobach reumatycznych. Wymusza to poszukiwanie nowych opcji terapeutycznych, ze szczególnym uwzględnieniem leków biologicznych. Forum Reumatol. 2015, tom 1, nr 1, 25–2

Nuclear Pedigree Criteria for the Identification of Individuals Suspected to be at Risk of an Inherited Predisposition to Renal Cancer

Renal clear cell carcinomas represent about 3% of all visceral cancers and account for approximately 85% of renal cancers in adults. Environmental and genetic factors are involved in the development of renal cancer. Although to date there are 19 hereditary syndromes described in which renal cell cancer may occur, only four syndromes with an unequivocal genetic predisposition to renal cell carcinoma have been identified: VHL syndrome (mutations in the VHL gene), hereditary clear cell carcinoma (translocations t(3:8), t(2:3)), hereditary papillary carcinoma (mutations in the MET protooncogene) and tuberous sclerosis (mutations in the TSC1 and TSC2 genes). Little is known genetically about the other forms of familial renal cell cancer. Since there is a growing awareness about the necessity of early intervention, clinical criteria have been developed that aid in the identification of hereditary forms of renal cancer. The aim of the current study was to identify minimal inclusion criteria so that nuclear pedigree families can be ascertained for risk assessment and/or kidney tumour screening. The results reveal that inclusion features described herein, such as (a) renal clear cell cancer diagnosed before 55 years of age, and (b) renal clear cell cancer and gastric cancer or lung cancer among first degree relatives, are useful in identifying suspected hereditary clear cell renal cancer patients

Śluzak prawego przedsionka u pacjenta z twardziną układową

The myxoma makes 80–90% of benign cardiac neoplasms. The first symptom in 50% of patients is an embolism resulting from relocation of tumor fragments or blood clots into the bloodstream. Elevated values of acute phase proteins in these patients are results of non-specific immune response to an antigen causing the disease. Interleukin 6 (IL-6) plays a role in this reaction, modifying inflammatory response by: influence on lymphocyte T differentiation, lymphocyte B to plasmocyte transformation, and stimulation of the liver to produce acute phase proteins. Elevated IL-6 is found in 80% of patients with diagnosed myxoma, which causes it to be an important marker in diagnostic and post-operational monitoring. The role in non-specific inflammatory response played by IL-6 in myxoma and autoimmune disorders was a cause of many diagnostic mistakes. Available literature does not suggest a coincidence of cardiac myxoma and systemic sclerosis. This is why we would like to present a case report, with special regard to correlation between IL-6 values and activity/stage of diagnosed disorders.Śluzak stanowi 80-90% łagodnych nowotworów serca. Pierwszym symptomem choroby u 50% pacjentów ze śluzakiem jest incydent zatorowy fragmentami guza lub skrzeplinami. Występujący wzrost wartości białek ostrej fazy u tych pacjentów jest związany z niespecyficzną reakcja immunologiczną. Często stwierdza się podwyższone stężenia białek ostrej fazy i immunoglobulin. Odpowiedzialną za to w głównej mierze jest interleukina 6, cytokina modyfikująca odpowiedź zapalną poprzez wpływ na różnicowanie się limfocytów T, transformację limfocytów B w plazmocyty oraz stymulacje białek ostrej fazy. Wzrost IL-6 obserwuje się u 80% pacjentów z rozpoznanym śluzakiem. Stanowi on w ten sposób ważny marker w dalszej obserwacji pooperacyjnej tej grupy pacjentów. Wspólne ogniwo jakim jest IL-6 w nieswoistej odpowiedzi immunologicznej o charakterze ogólnoustrojowym śluzaka i chorób o podłożu immunologicznym stał się źródłem pomyłek diagnostycznych. Nie opisywano do tej pory współistnienia twardziny układowej i śluzaka serca. Przedstawiamy przypadek chorego z twardziną układową i śluzakiem serca oraz korelację stężenia IL-6 z aktywnością rozpoznanych chorób

RISK FACTORS FOR EXTRA-ARTICULAR SIGNS IN SPONDYLOARTHRITIS

Background: There are data that development of different extra articular symptoms in seronegative spondyloarthropathies (SpA) is connected with elevated levels of different markers of inflammatory process Objectives: The aim the study was to assess risk factors of different extra-articular symptoms in SpA. Methods: We studied 287 SpA patients: 131 had AS, 110 had PsA, and 46 had SAPHO. We assessed extra-articular symptoms in all cases. In 191 SpA patients, we measured serum interleukin–6 (IL-6), interleukin–18 (IL-18), interleukin–23 (IL-23), endothelin-1 (ET-1) Results: In SpA patients as compared to healthy controls: 1. Increased serum levels of IL-6 (P=0.02), IL-23 (P=0.03), and Il-18 (P=0.0006) were associated with increased risk of acute anterior uveitis (AAU). 2. Increased serum levels IL-18 (P=0.03) were associated with an increased risk of inflammatory bowel disease (IBD). 3. Increased serum levels of IL-18 (P=0.0002) and decreased serum levels of ET-1 (P=0.006) were associated with increased risk of skin psoriasis. 4. Increased serum levels of IL-18 (P=0.0002) and decreased serum levels of ET-1 (P=0.008) were associated with increased risk of psoriatic onychopathy. 5. Increased serum levels of IL-18 (P=0.01) was associated with increased risk of palmo-plantar pustulosis. SpA patients with AAU (P=0.0008) and IBD (P=0.03) had higher VAS. SpA patients with skin psoriasis (P=0.001) and psoriatic onychopathy(P=0.006) had lower VAS. Conclusions: In SpA patients, increased serum IL-18 and decreased serum ET-1 were associated with an increased risk of extra-articular symptoms. Increased VAS was connected with AAU and IBD, decreased VAS – with skin psoriasis and psoriatic onychopathy

SAPHO SYNDROME – CLINICAL SYMPTOMS, IMAGING AND TREATMENT – BASED ON A GROUP OF POLISH PATIENTS

Background: Synovitis, acne, pustulosis, hyperostosis and osteitis (SAPHO) syndrome is a very rare disease presenting as a constellation of skin and osteoarticular symptoms. Objectives: We studied clinical symptoms, imaging and treatment in 52 Polish SAPHO patients. Methods: The following data were recorded: age, sex, disease duration, type of joint involvement, type of skin changes, bone scintigraphy results, HLA-B27, rheumatoid factor (RF), comorbidities and treatment. The patient’s pain due to the disease was assessed using a visual analogue scale (VAS). We also assessed the Bath Ankylosing Spondylitis Disease Activity Index (BASDAI). Results: SAPHO syndrome was more common in women with the mean age at diagnosis 50.0 years. All patients had a negative RF. 25% of 23 assessed patients had a positive HLA B-27 antigen. 88.5% of patients had palmoplantar pustulosis. Swelling and pain of sternoclavicular joints were the most common joint symptoms (present in 96.1 % of patients). Two patients (3.8%) had mandible involvement. Despite hypertension, the most prevalent comorbidities were hypothyroidism (9.8%), diabetes (9.8%) and depression (5.9%). DMARDs and antibiotics were useful in treatment. Conclusions: Mandible involvement is a rare manifestation of SAPHO syndrome. Increased incidence of autoimmune diseases and depression was observed. DMARDs and antibiotics were useful in treatment