Therapeutic plasma exchange (PLEX) is an adjunctive treatment for patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis and kidney involvement. Little is known about the effect of PLEX on early changes in kidney function. This post-hoc analysis of the PEXIVAS trial investigated the effects of PLEX on changes in kidney function within 12 months. PEXIVAS was a randomized controlled trial recruiting 691 patients with ANCA-associated glomerulonephritis, of whom 349 underwent PLEX and 342 received no-PLEX. The primary outcomes of this post hoc study of PEXIVAS were change in estimated glomerular filtration rate (eGFR) from baseline and recovery of kidney function (defined as eGFR increase of 15ml/min/1.73m2 or more). Baseline eGFR was 21.7 ± 20.3 and 20.6 ± 18.7 ml/min/1.73m2 in the PLEX and no-PLEX groups, respectively. Mean improvements in eGFR at weeks two, four, and eight after initiation of therapy were greater for the PLEX vs. the no-PLEX groups. The greatest significant difference in recovery of kidney function in the PLEX compared to the no-PLEX groups was at week four (relative risk (RR): 1.41; 95% confidence interval:1.09-1.82). Increased eGFR or recovery of kidney function at week four were significantly associated with lower risk for end-stage kidney disease at week 52 (RR: 0.96: 0.95-0.97, and RR: 0.29: 0.16-0.52; respectively). Neither changes in eGFR nor recovery of kidney function differed by reduced- compared to standard-dose glucocorticoid group. Overall, our study indicates that PLEX improves early kidney function in patients with ANCA-associated glomerulonephritis.

• MeSH
• ANCA-asociované vaskulitidy * patofyziologie   terapie   farmakoterapie   komplikace   imunologie   diagnóza   MeSH
• dospělí MeSH
• glomerulonefritida * patofyziologie   imunologie   terapie   krev   MeSH
• glukokortikoidy * terapeutické užití   aplikace a dávkování   MeSH
• hodnoty glomerulární filtrace * MeSH
• ledviny * patofyziologie   účinky léků   MeSH
• lidé středního věku MeSH
• lidé MeSH
• obnova funkce MeSH
• senioři MeSH
• výměna plazmy * MeSH
• výsledek terapie MeSH
• Check Tag
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• multicentrická studie MeSH
• randomizované kontrolované studie MeSH

• Klíčová slova
• doporučení KDIGO,
• MeSH
• ANCA-asociované vaskulitidy farmakoterapie   imunologie   MeSH
• glomerulonefritida * diagnóza   farmakoterapie   klasifikace   MeSH
• IgA nefropatie farmakoterapie   imunologie   MeSH
• lidé MeSH
• nefritida při lupus erythematodes farmakoterapie   imunologie   MeSH
• randomizované kontrolované studie jako téma MeSH
• směrnice pro lékařskou praxi jako téma MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• přehledy MeSH

V posledních letech je věnována pozornost pacientům s intersticiálním plicním postižením (IPP) při systémových chorobách pojiva. Odpovídá tomu i množství doporučených postupů zabývajících se touto problematikou. Většina hodnotí efekt již známých molekul v managementu IPP. V průběhu roku 2024 nebyla publikována žádná klinická studie, která by obohatila portfolio léčebných možností novou molekulou. Nově byly publikovány doporučené postupy managementu a léčby plicních alveolárních proteinóz. Ukazuje se, že u nemocných s idiopatickou plicní fibrózou, kteří utrpí akutní exacerbací, lze léčbu vysokou dávkou kortikosteroidů považovat spíše za škodlivou než přínosnou pro pacienta. Dlouhodobá domácí oxygenoterapie by měla být nadále vyhrazena pouze pro pacienty s IPP, kteří splňují indikační kritéria pro tuto terapii. Vliv na zmírnění námahové dušnosti v běžném životě nemocných nebyl prokázán. Stejně tak nebyl prokázán benefit léčby opioidy k mírnění symptomů. Národní doporučené postupy věnované této problematice jsou postupně aktualizovány a jsou dostupné na stránkách České pneumoftizeologické společnosti.

In recent years, attention has been paid to patients with connective tissue diseases and interstitial lung disease (ILD). This is reflected in the number of recently published guidelines dealing with this issue. Most of them evaluate the effect of already known molecules on the management of ILDs. During 2024, no clinical study was published that would enrich the portfolio of treatment options with a new molecule. Guidelines for the management and treatment of pulmonary alveolar proteinosis were recently published. It turns out that in patients with idiopathic pulmonary fibrosis who suffer acute exacerbation, treatment with high doses of corticosteroids can be considered more harmful than beneficial to the patient. Home oxygen therapy should continue to be reserved only for patients with ILD who meet the indication criteria for this therapy. The effect on alleviating exertional dyspnea in the daily life of patients has not been proven. Similarly, the benefit of opioid treatment to alleviate symptoms has not been proven. National guidelines dedicated to this issue are gradually updated and are available on the website of Czech Pneumological and Phthiseological Society.

• MeSH
• intersticiální plicní nemoci * terapie   MeSH
• lidé MeSH
• nemoci pojiva terapie   MeSH
• plicní alveolární proteinóza terapie   MeSH
• směrnice pro lékařskou praxi jako téma MeSH
• vaskulitida terapie   MeSH
• Check Tag
• lidé MeSH

Autorka v príspevku uvádza kazuistiku pacienta s pokročilou granulomatózou s polyangitídou, ktorý bol privezený po úspešnej kardiopulmonálnej resuscitácii v rámci infarktu myokardu v ťažkom kardiogénnom šoku a vyžadoval umelú pľúcnu ventiláciu. Autorka sa snaží poukázať na závažnosť základného ochorenia, s ktorým sa v súčasnej dobe stretávame čoraz častejšie. Zároveň vyzdvihuje dôležitosť primárnej zdravotnej starostlivosti, ktorá prispieva k správnej diagnostike a liečbe ochorenia.

The author presents a case study of a patient with advanced granulomatosis with polyangiitis, who was admitted after successful cardiopulmonary resuscitation due to myocardial infarction in severe cardiogenic shock and required mechanical ventilation. The author aims to high light the severity of the underlying disease, which is being encountered increasingly often in contemporary practice. At the same time, the author emphasizes the importance of primary healthcare in contributing to the accurate diagnosis and treatment of the disease

• MeSH
• granulomatóza s polyangiitidou diagnostické zobrazování   etiologie   klasifikace   patofyziologie   terapie   MeSH
• infarkt myokardu ošetřování   terapie   MeSH
• kardiogenní šok MeSH
• ošetřovatelská péče o pacienty v kritickém stavu MeSH
• péče o pacienty v kritickém stavu MeSH
• plicní ventilace MeSH
• výsledek terapie MeSH
• Publikační typ
• kazuistiky MeSH

Čtyřletý chlapec s dosud bezvýznamnou anamnézou byl přijat pro krční lymfadenopatii a febrilie. Od 3. dne hospitalizace výsev drobnopapulozního exantému a bolesti břicha, pro které byla nutná laparoskopická revize, nález flegmonózní apendicitidy, provedena apendektomie. Pro suspektní toxoalergický exantém byla opakovaně měněna antibiotická léčba. I přes širokospektrá antibiotika stále stoupaly zánětlivé markery, transaminázy, lymfadenopatie výrazněji neregredovala, exantém a febrilie přetrvávaly, objevila se konjunktivitida, rozpraskané rty, artralgie. Diferenciálně diagnosticky zvažována EB viróza, parainfekční exantém, vysloveno podezření na Kawasakiho syndrom. I přes negativní nález při kardiologickém vyšetření zahájena standardní terapie Kawasakiho syndromu - jednou dávkou imunoglobulinu (IVIG) a kyselinou acetylsalicylovou (ASA). Po podání IVIG promptně poklesly teploty, regredoval exantém, lymfadenopatie, normalizoval se laboratorní nález. Uzavíráme jako Kawasakiho syndrom.

A 4-year-old boy with a previously unremarkable medical history was admitted for cervical lymphadenopathy and fever. From the 3rd day of hospitalization a small-papu­lous exanthema and abdominal pain for which laparoscopic revision was necessary, phlegmonous appendicitis was found, and appendectomy was performed. Antibiotic treatment was repeatedly changed for suspected toxoallergic exanthema. Despite broad-spectrum antibiotics, inflammatory markers, transaminases continued to rise, lymphadenopathy did not significantly regress, exanthema, febrile persisted, conjunctivitis, chapped lips, arthralgia appeared. Differential diagnostic consideration was given to EB virosis, parainfectious exanthema, Kawasaki syndrome was suspected. Despite of negative findings on cardiological examination the treatment with one dose of intravenous immunoglo­bulins (IVIG) and acetylsalicylic acid (ASA) was administered. After administration of IVIG followed prompt decrease in temperature, regression of exanthema, nodal syndrome, normalization of laboratory findings. We concluded as Kawasaki syndrome.

• MeSH
• apendicitida chirurgie   diagnóza   etiologie   MeSH
• Aspirin farmakologie   terapeutické užití   MeSH
• diferenciální diagnóza MeSH
• exantém diagnóza   etiologie   MeSH
• horečka neznámého původu diagnóza   etiologie   MeSH
• intravenózní imunoglobuliny farmakologie   terapeutické užití   MeSH
• Kawasakiho syndrom * diagnóza   epidemiologie   farmakoterapie   krev   MeSH
• lidé MeSH
• lymfadenopatie diagnóza   etiologie   MeSH
• předškolní dítě MeSH
• Check Tag
• lidé MeSH
• mužské pohlaví MeSH
• předškolní dítě MeSH
• Publikační typ
• kazuistiky MeSH
• práce podpořená grantem MeSH

• MeSH
• ANCA-asociované vaskulitidy komplikace   terapie   MeSH
• glomerulonefritida * etiologie   terapie   MeSH
• IgA nefropatie farmakoterapie   MeSH
• lidé MeSH
• nefritida při lupus erythematodes farmakoterapie   MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• přehledy MeSH

BACKGROUND: To determine differences in the blood innate gene expression signatures of systemic lupus erythematosus (SLE) patients across various organ manifestations and disease activity, with a focus on lupus nephritis (LN) and central nervous system (CNS) involvement. METHODS: Toll-like receptor family (TLR 1-10) mRNA expression was investigated in peripheral blood mononuclear cells from patients with SLE (n = 74) and healthy controls (n = 34). We compared patients with histologically confirmed active LN or neuropsychiatric systemic lupus erythematosus (NPSLE) with patients without these symptoms. The expression of TLR mRNA was determined by RT‒qPCR using a high-throughput SmartChip Real-Time-qPCR system (WaferGen). Multivariate analysis and nonparametric statistics were used for data analysis to assess the associations between TLRs and disease activity and severity. RESULTS: TLR4 (0.044 vs. 0.081, p = 0.012) was upregulated and TLR10 (0.009 vs. 0.006, p = 0.0007) was downregulated in the whole cohort of SLE patients compared to healthy controls. A comparison of the active LN group with participants without kidney involvement revealed increased expression of TLR2 (0.078 vs. 0.03, p = 0.009), and TLR5 (0.035 vs. 0.017, p = 0.03). Moreover, a significant difference was observed in TLR9 expression between inactive LN and the control group (0.014 vs. 0.009, p = 0.01), together with borderline correlation in TLR2 expression (0.04 vs. 0.03, p = 0.06). Receiver operating characteristic (ROC) curve analysis revealed that TLR1 and TLR2 expression were the best potential diagnostic markers for active LN. The NPSLE group showed upregulation of TLR1 (0.088 vs. 0.048, p = 0.01), TLR4 (0.173 vs. 0.066, p = 0.0003) and TLR6 (0.087 vs. 0.036, 0.007). Our correlation analysis supported the close relationships among the expression of individual TLRs in the whole lupus cohort and its subgroups. CONCLUSION: Our study revealed differences in TLR expression between a lupus cohort and healthy controls. Additionally, our analysis provides insight into specific TLR expression in cases with severe organ manifestations, such as LN and NPSLE. The multiple mutual relationships of TLRs demonstrate the activation of innate immunity in SLE and suggest promising targets for future therapies or diagnostics.

• MeSH
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mladý dospělý MeSH
• nefritida při lupus erythematodes * genetika   krev   MeSH
• systémový lupus erythematodes krev   genetika   MeSH
• toll-like receptory * genetika   biosyntéza   MeSH
• vaskulitida centrálního nervového systému při lupus erythematodes * krev   genetika   MeSH
• Check Tag
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mladý dospělý MeSH
• mužské pohlaví MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH

BACKGROUND: The neurofilament light chain (NfL) in cerebrospinal fluid (CSF) and serum as a marker of neuronal damage may be a potential biomarker of neuropsychiatric involvement in SLE (NPSLE). METHODS: 80 patients with SLE were included.We obtained paired serum and CSF samples from 48 patients (NPSLE n=32, non-NPSLE n=16) and 31 controls. The serum and CSF levels of NfL were determined using ELISA. RESULTS: Patients with NPSLE demonstrated significantly higher levels of serum NfL compared with the non-NPSLE group (mean 31.68±36.63 pg/mL vs mean 16.75±12.48 pg/mL, respectively, p<0.05) and with controls (mean 10.74±4.36 pg/mL, p<0.01). Notably, CSF NfL concentrations in patients with NPSLE showed an upward trend (mean 1600±2852 pg/mL) in contrast to non-NPSLE patients (mean 393.4±191.9 pg/mL) and controls (mean 509.7±358.5 pg/mL). Furthermore, a positive correlation was observed between serum and CSF NfL levels in patients with NPSLE (R=0.8686, p<0.01). Elevated serum triacylglycerol concentrations, C reactive protein and organ damage were linked to increased serum (p=0.002; p<0.001; p=0.036) and CSF (p=0.008; p=0.007; p<0.001) NfL concentrations. In addition, we established a significant correlation between intrathecal NfL concentrations and interleukin-6 levels in the CSF of patients with NPSLE (R=0.5118, p<0.05). CONCLUSION: The serum NfL levels may be a readily available marker of neuropsychiatric involvement in SLE.

• MeSH
• biologické markery * krev   mozkomíšní mok   MeSH
• dospělí MeSH
• ELISA MeSH
• interleukin-6 krev   mozkomíšní mok   MeSH
• lidé středního věku MeSH
• lidé MeSH
• mladý dospělý MeSH
• neurofilamentové proteiny * krev   mozkomíšní mok   MeSH
• průřezové studie MeSH
• studie případů a kontrol MeSH
• vaskulitida centrálního nervového systému při lupus erythematodes * krev   mozkomíšní mok   MeSH
• Check Tag
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mladý dospělý MeSH
• mužské pohlaví MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH

• MeSH
• dospělí MeSH
• granulomatóza s polyangiitidou * diagnóza   farmakoterapie   komplikace   MeSH
• lidé MeSH
• prednison terapeutické užití   MeSH
• progrese nemoci MeSH
• recidiva MeSH
• rituximab terapeutické užití   MeSH
• skleritida * etiologie   farmakoterapie   patologie   MeSH
• Check Tag
• dospělí MeSH
• lidé MeSH
• ženské pohlaví MeSH
• Publikační typ
• kazuistiky MeSH

SIGNIFICANCE STATEMENT: Reliable prediction tools are needed to personalize treatment in ANCA-associated GN. More than 1500 patients were collated in an international longitudinal study to revise the ANCA kidney risk score. The score showed satisfactory performance, mimicking the original study (Harrell's C=0.779). In the development cohort of 959 patients, no additional parameters aiding the tool were detected, but replacing the GFR with creatinine identified an additional cutoff. The parameter interstitial fibrosis and tubular atrophy was modified to allow wider access, risk points were reweighted, and a fourth risk group was created, improving predictive ability (C=0.831). In the validation, the new model performed similarly well with excellent calibration and discrimination ( n =480, C=0.821). The revised score optimizes prognostication for clinical practice and trials. BACKGROUND: Reliable prediction tools are needed to personalize treatment in ANCA-associated GN. A retrospective international longitudinal cohort was collated to revise the ANCA renal risk score. METHODS: The primary end point was ESKD with patients censored at last follow-up. Cox proportional hazards were used to reweight risk factors. Kaplan-Meier curves, Harrell's C statistic, receiver operating characteristics, and calibration plots were used to assess model performance. RESULTS: Of 1591 patients, 1439 were included in the final analyses, 2:1 randomly allocated per center to development and validation cohorts (52% male, median age 64 years). In the development cohort ( n =959), the ANCA renal risk score was validated and calibrated, and parameters were reinvestigated modifying interstitial fibrosis and tubular atrophy allowing semiquantitative reporting. An additional cutoff for kidney function (K) was identified, and serum creatinine replaced GFR (K0: <250 μ mol/L=0, K1: 250-450 μ mol/L=4, K2: >450 μ mol/L=11 points). The risk points for the percentage of normal glomeruli (N) and interstitial fibrosis and tubular atrophy (T) were reweighted (N0: >25%=0, N1: 10%-25%=4, N2: <10%=7, T0: none/mild or <25%=0, T1: ≥ mild-moderate or ≥25%=3 points), and four risk groups created: low (0-4 points), moderate (5-11), high (12-18), and very high (21). Discrimination was C=0.831, and the 3-year kidney survival was 96%, 79%, 54%, and 19%, respectively. The revised score performed similarly well in the validation cohort with excellent calibration and discrimination ( n =480, C=0.821). CONCLUSIONS: The updated score optimizes clinicopathologic prognostication for clinical practice and trials.

• MeSH
• ANCA-asociované vaskulitidy * diagnóza   MeSH
• atrofie MeSH
• fibróza MeSH
• kreatinin MeSH
• ledviny MeSH
• lidé středního věku MeSH
• lidé MeSH
• longitudinální studie MeSH
• protilátky proti cytoplazmě neutrofilů * MeSH
• retrospektivní studie MeSH
• rizikové faktory MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH