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Reumatologiczne manifestacje pierwotnych niedoborów odporności

100%

Ziętkiewicz M., Zdrojewski Z.

Alergia Astma Immunologia - przegląd kliniczny

2018

vol. 23

issue 1

40-45

Primary immunodeficiency (PID) constitutes the group of over 300 disorders of varied clinical picture. It is estimated that there might be approximately six million people suffering from PID worldwide. In the course of primary immunodeficiency not only recurring infections may occur, but also autoimmune disorders. This group also includes a number of diseases which are of interest for rheumatologists, such as connective tissue diseases (e.g. systemic lupus erythematosus). Moreover, the increased proneness to infections might be the cause of the occurrence of septic or reactive arthritis. In the course of several PID no recurring infections occur. In the light of the data published in 2017, based on the analysis of the fortunes of 2183 French patients suffering from primary immunodeficiency, rheumatic diseases might constitute the fourth largest group in terms of the disorders which are the most frequent in the course of PID. The only more frequent groups are autoimmune cytopenia, digestive system diseases and skin diseases (with the exception of vasculitis). The thesis presents the connections between the occurrence of systemic lupus erythematosus, arthritis, vasculitis and dermatomyositis, and the selected primary immunodeficiencies.

Pierwotne niedobory odporności (PNO) stanowią grupę ponad 300 jednostek o zróżnicowanym obrazie klinicznym. Szacuje się, że na świecie może żyć około sześć milionów ludzi z PNO. W przebiegu pierwotnych niedoborów odporności mogą występować nie tylko nawracające infekcje ale również schorzenia o podłożu autoimmunologicznym. Do tej grupy zalicza się również szereg chorób będących w kręgu zainteresowań reumatologów takich jak układowe choroby tkanki łącznej (np. toczeń układowy rumieniowaty). Dodatkowo zwiększona skłonność do zakażeń może być przyczyną występowania septycznych bądź odczynowych zapaleń stawów. W przebiegu niektórych PNO w ogóle nie występują nawracające zakażenia. W świetle opublikowanych w 2017 roku danych, opracowanych na podstawie analizy losów 2183 francuskich pacjentów z pierwotnymi niedoborami odporności, choroby reumatyczne mogą stanowić czwartą pod względem częstości występowania grupę schorzeń występujących w przebiegu PNO. Częściej występowały tylko cytopenie o podłożu autoimmunologicznym, choroby przewodu pokarmowego oraz skóry (z wyłączeniem zapaleń naczyń). W pracy przedstawiamy związki pomiędzy występowaniem tocznia układowego rumieniowatego, zapaleniami stawów, zapaleniem naczyń i zapaleniem skórno-mięśniowym a wybranymi pierwotnymi niedoborami odporności.

Atorvastatin improves tubular status in non-diabetic patients with chronic kidney disease - placebo controlled, randomized, cross-over study

52%

Renke M., Tylicki L., Rutkowski P., Neuwelt A., Larczyński W., Ziętkiewicz M., Aleksandrowicz E., Łysiak-Szydłowska W., Rutkowski B.

Acta Biochimica Polonica

2010

vol. 57

issue 4

547-552

Background. There is evidence that dyslipidemia is associated with chronic kidney disease (CKD) and it has been implicated in the progression of renal damage. Optimal management of dyslipidemia should therefore lead to renal benefits. A number of experimental models demonstrate a beneficial effect of statins in ameliorating renal damage. However, the exact mechanism by which statins protect against renal damage remains unclear. Methods. In a placebo-controlled, randomized, cross-over study we evaluated the influence of atorvastatin (ATO) 40 mg/day added to the renin-angiotensin-aldosterone systeme (RAAS) blockade on proteinuria and surrogate biomarkers of tubular damage or injury in 14 non-diabetic patients with proteinuria (0.4-1.8 g per 24 h) with normal or declined kidney function (eGFR 55-153 ml/min). In the eight-week run-in period, therapy using angiotensin converting enzyme inhibitors (ACEI) and/or angiotensin II subtype 1 receptor antagonists (ARB) was adjusted to achieve a blood pressure below 130/80 mm Hg. Next, patients were randomly assigned to one of two treatment sequences: ATO/washout/placebo or placebo/washout/ATO. Clinical evaluation and laboratory tests were performed at the randomization point and after each period of the study. The primary end point of this study was a change in proteinuria measured as 24-h urine protein excretion (DPE). Secondary end points included urine N-acetyl-β-d-glucosaminidase (NAG) and α1-microglobulin (α1m) excretion. Results. The ATO therapy significantly reduced urine excretion of α1m (p=0.033) and NAG (p=0.038) as compared to placebo. There were no differences in proteinuria, blood pressure, eGFR and serum creatinine between the ATO and placebo groups. Conclusion. Atorvastatin treatment is safe and improves biomarkers of tubular damage or injury in non-diabetic patients with CKD.

Effect of pentoxifylline on proteinuria, markers of tubular injury and oxidative stress in non-diabetic patients with chronic kidney disease - placebo controlled, randomized, cross-over study

52%

Renke M., Tylicki L., Rutkowski P., Knap N., Ziętkiewicz M., Neuwelt A., Aleksandrowicz E., Łysiak-Szydłowska W., Woźniak M., Rutkowski B.

Acta Biochimica Polonica

2010

vol. 57

issue 1

119-123

Background: Inhibition of the renin-angiotensin-aldosterone system (RAAS) with angiotensin converting enzyme inhibitors (ACEI) and/or angiotensin II subtype 1 receptor antagonists (ARB) is a common strategy used in the management of patients with chronic kidney disease (CKD). However, there is no universal therapy that can stop progression of CKD. Pentoxifylline (PTE) is a non-specific phosphodiesterase inhibitor with anti-inflammatory properties. It has been reported to have promising effects in CKD treatment. Methods: In a placebo-controlled, randomized, cross-over study we evaluated the influence of PTE (1200 mg/day) added to RAAS blockade on proteinuria, surrogate markers of tubular injury and oxidative stress-dependent products in 22 non-diabetic patients with proteinuria (0.4-4.3 g per 24h) with normal or declined kidney function [eGFR 37-178 mL/min]. In an eight-week run-in period, therapy using ACEI and/or ARB was adjusted to achieve a blood pressure below 130/80 mm Hg. Next, patients were randomly assigned to one of two treatment sequences: PTE/washout/placebo or placebo/washout/PTE. Clinical evaluation and laboratory tests were performed at the randomization point and after each period of the study. Results: The PTE therapy reduced proteinuria (by 26%) as compared to placebo. There were no differences in α1-microglobulin, urine excretion of N-acetyl-β-d-glucosaminidase (NAG), hsCRP, the urinary excretion of 15-F2t-isoprostane, blood pressure (BP), eGFR and serum creatinine between the PTE and placebo groups. Conclusion: Pentoxifylline may decrease proteinuria in non-diabetic patients with CKD.

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2 Acta Biochimica Polonica

1 Alergia Astma Immunologia - przegląd kliniczny

1 2018

2 2010

3 Ziętkiewicz M.

2 Aleksandrowicz E.

2 Neuwelt A.

2 Renke M.

2 Rutkowski B.

2 Rutkowski P.

2 Tylicki L.

2 Łysiak-Szydłowska W.

1 Knap N.

1 Larczyński W.

1 Woźniak M.

1 Zdrojewski Z.

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Reumatologiczne manifestacje pierwotnych niedoborów odporności

Atorvastatin improves tubular status in non-diabetic patients with chronic kidney disease - placebo controlled, randomized, cross-over study

Effect of pentoxifylline on proteinuria, markers of tubular injury and oxidative stress in non-diabetic patients with chronic kidney disease - placebo controlled, randomized, cross-over study