ENWEndNote BIBJabRef, Mendeley RISPapers, Reference Manager, RefWorks, Zotero AMA Włudarczyk A, Biedroń G, Wójcik K, et al. ANCA-associated vasculitis patients treated in Polish intensive care units – retrospective characteristics based on the POLVAS registry. Anaesthesiology Intensive Therapy. 2020;52(4):281-286. doi:10.5114/ait.2020.100047. APA Włudarczyk, A., Biedroń, G., Wójcik, K., Zdrojewski, Z., Masiak, A., & Czuszyńska, Z. et al. (2020). ANCA-associated vasculitis patients treated in Polish intensive care units – retrospective characteristics based on the POLVAS registry. Anaesthesiology Intensive Therapy, 52(4), 281-286. https://doi.org/10.5114/ait.2020.100047 Chicago Włudarczyk, Anna, Grzegorz Biedroń, Krzysztof Wójcik, Zbigniew Zdrojewski, Anna Masiak, Zenobia Czuszyńska, and Maria Majdan et al. 2020. "ANCA-associated vasculitis patients treated in Polish intensive care units – retrospective characteristics based on the POLVAS registry". Anaesthesiology Intensive Therapy 52 (4): 281-286. doi:10.5114/ait.2020.100047. Harvard Włudarczyk, A., Biedroń, G., Wójcik, K., Zdrojewski, Z., Masiak, A., Czuszyńska, Z., Majdan, M., Jeleniewicz, R., Krajewska, M., Kusztal, M., Brzosko, M., Brzosko, I., Dębska-Ślizień, A., Storoniak, H., Tłustochowicz, W., Kur-Zalewska, J., Rydzewski, A., Madej, M., Hawrot-Kawecka, A., Stasiek, M., Kucharz, E., Musial, J., and Szczeklik, W. (2020). ANCA-associated vasculitis patients treated in Polish intensive care units – retrospective characteristics based on the POLVAS registry. Anaesthesiology Intensive Therapy, 52(4), pp.281-286. https://doi.org/10.5114/ait.2020.100047 MLA Włudarczyk, Anna et al. "ANCA-associated vasculitis patients treated in Polish intensive care units – retrospective characteristics based on the POLVAS registry." Anaesthesiology Intensive Therapy, vol. 52, no. 4, 2020, pp. 281-286. doi:10.5114/ait.2020.100047. Vancouver Włudarczyk A, Biedroń G, Wójcik K, Zdrojewski Z, Masiak A, Czuszyńska Z et al. ANCA-associated vasculitis patients treated in Polish intensive care units – retrospective characteristics based on the POLVAS registry. Anaesthesiology Intensive Therapy. 2020;52(4):281-286. doi:10.5114/ait.2020.100047.
(1) Background: Impaired nutritional status in systemic sclerosis (SSc) is prevalent. (2) Objective: This study aimed to identify pre-cachexia and malnutrition in SSc patients and to estimate the effectiveness of a high-protein oral nutritional supplement (ONS) in improving their nutritional status. (3) Materials and methods: The SSc population comprised 56 patients and a control group of 49 healthy persons. After a baseline clinical evaluation, bioelectrical impedance analysis (BIA), and laboratory tests, SSc patients were divided into well-nourished, pre-cachectic, and malnourished categories. SSc patients with a nutritional disbalance received a high-protein ONS once daily for 3 months. Patients were reassessed at 3 and 12 months after inclusion in the study. (4) Results: SSc patients, in comparison to the control group, had a significantly lower seven-point SGA value [6(0) vs. 7(1),
Systemic manifestations are common in primary Sjögren's syndrome (pSS) and play a major role in the prognosis. Neurological complications may affect both the peripheral (PNS) and central nervous system (CNS). The incidence varies from several to several dozen percent. The course of the disease and the severity of the symptoms may be mild and self-limiting or progressive, leading to permanent neurological deficits. It is worth remembering that the symptoms of nervous system involvement may precede the onset of symptoms of dryness, and diagnosis of pSS may be delayed after a certain duration of neurological symptoms.
Objectives:
The aim of the study was to determine the prevalence of PNS involvement among symptomatic and asymptomatic patients with the diagnosis of pSS in our University Clinical Centre.
Methods:
We studied a consecutive group of fifty unselected patients aged from 33 to 74 years (mean 55.8 years). All patients fulfilled the criteria for the diagnosis of pSS. Additional connective tissue diseases and diabetes were the exclusion criteria. Disease activity was evaluated according to the EULAR Sjögren's syndrome disease activity index (ESSDAI). All patients underwent clinical neurological examination and nerve conduction studies (NCS) of nine peripheral nerves. For the classification of polyneuropathies the ESTEEM (European Standardized Telematic tool to Evaluate Electrodiagnostic Methods) guidelines were used. Clinical examination and nerve conduction study were performed and evaluated by one certified neurologist.
Results:
Of our 50 patients, 48 were female with a mean (±SD) age 53.6±10.5 years and mean disease duration 7.9±5.3 years. Two were male with mean age 43.7±25.8 years and mean disease duration 5.7±0.6 years. The mean age at diagnosis was 50.4±14 years. 23 (46%) patients fulfilled the criteria for the diagnosis of neuropathy. The most common PNS manifestation was sensorimotor neuropathy 11/23 (47%), mononeuropathy was present in 6/23 (26%) patients, pure axonal sensory neuropathy was present in 1/23 (4.3%) patient, axonal motor neuropathy in 1/23 (4.3%), SFN in 1/23 (4.3%) and cranial nerve involvement was present in 4/23 (17.4%) (one of the patients had both cranial and sensorimotor neuropathy). Neurological symptoms preceded the diagnosis of pSS in 8 (35%) of 23 PNS+ patients. The frequency of following symptoms and extraglandular manifestations was significantly higher in PNS+ compared to PNS- patients: salivary gland enlargement (74% vs 44% p<0,05), respiratory tract involvement (65% vs 37% p<0,05) and lymphadenopathy (61% vs 19% p<0,05). The mean ESSDAI in patients with and without neurological involvement was 7±6,82 and 4,7±4,58 respectively (p=0.245). In this subgroup the use of cyclophosphamide, due to extraglandular manifestations, was increased (p<0,05).
Conclusion:
We found that PNS involvement is a common extraglandular manifestation of pSS (46% in our group). Sensorimotor neuropathies were most frequent. Involvement of the PNS seems frequent but remains underestimated. NCS is a non-invasive test which might be useful at diagnosis and follow-up. Guidelines for the diagnosis and treatment of peripheral neuropathies in patients with pSS are needed.
Objectives Illness perception is a cognitive representation influencing physical and psychological functioning and adherence in patients with rheumatic disease. Studies exploring illness perception in systemic lupus erythematosus (SLE) are still scarce and none of them have investigated factors determining illness perception. We aimed to assess illness perception and to identify psychological, clinical and sociodemographic factors that might influence illness perception in SLE. Methods The study involved 80 patients with SLE (87.5% women, mean age 41.56 years). The Brief Illness Perception Questionnaire, State Trait Anxiety Inventory, Beck Depression Inventory, Pittsburgh Sleep Quality Index, Visual Analogue Scale-Pain and Fatigue Severity Scale were used. Clinical and sociodemographic data were collected via structured interview and medical files review. Results Illness perception was significantly positively correlated with anxiety, depression, sleep quality, fatigue and pain while it was not related to age, education, steroid treatment, disease duration and activity (SLEDAI) or organ damage (SLICC/ACR). Regression analysis revealed that state anxiety and depression explained 43% of illness perception variance. Cluster analysis identified three patient groups among which the middle-aged group had the most negative illness perception, the highest levels of anxiety, depression, pain and fatigue, and the poorest sleep quality. Conclusions The study has proved a significant relationship between negative illness perception and anxiety and depression. Patients reporting fatigue, poor sleep and pain might have special needs in terms of psychological intervention focused on negative illness perception and distress symptoms. Multidisciplinary care in managing SLE seems to be of great importance.
MCTD is a rare connective tissue disease with many controversies regarding its recognition, clinical course and assessment. The clinical expression of MCTD is the consequence of its complex immunopathology. The chronic systemic inflammation leads to blood vessel destruction and serious internal organ damage. Therefore, early detection of vascular involvement could play an essential role in the diagnostic procedures of MCTD. Nailfold capillaroscopy (NFC) is widely used as a simple, non-invasive technique for investigating microvascular involvement in rheumatic diseases [1].
Objectives
To describe the course of MCTD and its relation to nailfold capillaroscopy patterns.
Methods
We analyzed clinical picture and nailfold capillaroscopy patterns (NFC) in 69 patients (pts) with MCTD recognized according to Kasukawa criteria. Clinical data achieved on the day of examination were compared with the data from beginning of the disease collected retrospectively. NFC was performed on the day of examination and analysed by one investigator. NFC changes were classified as normal (grade 0), mild (grade 1), moderate and severe (grade 3, 4). We assessed correlation of NFC changes with clinical symptoms using Spearman’s rank correlation. Study was accepted by Local Ethics Committee.
Results
Median of MCTD pts age was 45 years (16 - 66), median of disease duration: 117,8 months (10-420). The beginning of disease was mainly subacute (65%), and its course was more frequently chronically progressive (63,8%). Among features with frequency diminishing from the beginning of disease to follow-up were: arthropathy, puffy hands, SLE –like skin involvement, myositis, serositis and laboratory abnormalities: haematological, hypergammaglobulinaemia and elevation of inflammatory indicators- ESR, CRP. Features with increasing prevalence in the course of the disease were: scleroderma-like skin involvement, interstitial lung disease, secondary Sjögren’s syndrome, pulmonary arterial hypertension. A Raynaud’s phenomenon was observed with similar frequency at disease onset and follow-up. Median of ANA titer was 1:5 840 (1:80 - 1:40 960). Anti-U1 RNP revealed in 98,5% of pts, anti-68kD – in 79,4%, anti-A in 88,2% and anti-C in 80,9% of pts. A NFC abnormality grade 1 was detected in 58% pts, grade 2 and 3 in 17.6%. No abnormalities were observed in 23.5% pts (grade 0). We did not find any correlation between NFC pattern and any clinical and serological features.
Conclusions
Our data confirm that the beginning of MCTD is subacute with mild and moderate symptoms. Later, with disease course severe organ involvement and Sjögren’s syndrome occurred, what justifies regular screening and close monitoring of the MCTD patients. Capillaroscopy abnormalities are independent feature but, opposite to systemic sclerosis, they did not correlate with severity of organ involvement.
References
Furtado RN, Pucinelli MC, Cristo VV et al. Sleroderma-like nailfold capillaroscopic abnormalities are assiciated with anti-U1-RNP antibodies and Raynaud’s phenomenon in SLE patients. Lupus 2002;11:35-41.
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are rare small to medium-size vessel systemic diseases. As their clinical picture, organ involvement, and factors influencing outcome may differ between countries and geographical areas, we decided to describe a large cohort of Polish AAV patients coming from several referral centers—members of the Scientific Consortium of the Polish Vasculitis Registry (POLVAS). We conducted a systematic multicenter retrospective study of adult patients diagnosed with AAV between Jan 1990 and Dec 2016 to analyze their clinical picture, organ involvement, and factors influencing outcome. Patients were enrolled to the study by nine centers (14 clinical wards) from seven Voivodeships populated by 22.3 mln inhabitants (58.2% of the Polish population). Participating centers included 625 AAV patients into the registry. Their distribution was as follows: 417 patients (66.7%) with GPA, 106 (17.0%) with MPA, and 102 (16.3%) with EGPA. Male-to-female ratios were almost 1:1 for GPA (210/207) and MPA (54/52), but EGPA was twice more frequent among women (34/68). Clinical manifestations and organ involvement were analyzed by clinical phenotype. Their clinical manifestations seem very similar to other European countries, but interestingly, men with GPA appeared to follow a more severe course than the women. Fifty five patients died. In GPA, two variables were significantly associated with death: permanent renal replacement therapy (PRRT) and respiratory involvement (univariate analysis). In multivariate analysis, PRRT (OR = 5.3; 95% confidence interval (CI) = 2.3–12.2), respiratory involvement (OR = 3.2; 95% CI = 1.06–9.7), and, in addition, age > 65 (OR = 2.6; 95% CI = 1.05–6.6) were independently associated with death. In MPA, also three variables were observed to be independent predictors of death: PRRT (OR = 5.7; 95% CI = 1.3–25.5), skin involvement (OR = 4.4; 95% CI = 1.02–19.6), and age > 65 (OR = 6.3; 95% CI = 1.18–33.7). In this first multicenter retrospective study of the Polish AAV patients, we have shown that their demographic characteristics, disease manifestations, and predictors of fatal outcome follow the same pattern as those from other European countries, with men possibly suffering from more severe course of the disease.
The study evaluates the psychometric properties of a Polish translation of the Brief Illness Perception Questionnaire. A total of 276 patients with chronic conditions (58.7% women) completed the Brief Illness Perception Questionnaire and the Hospital Anxiety and Depression Scale. The internal consistency of the Polish Brief Illness Perception Questionnaire measured with Cronbach's alpha was satisfactory (α = 0.74). Structural validity was demonstrated by significant inter-correlations between the Brief Illness Perception Questionnaire components. Discriminant validity was supported by the fact that the Brief Illness Perception Questionnaire enables patients with various conditions to be differentiated. Significant correlations were found between Brief Illness Perception Questionnaire and depression and anxiety levels. The Polish Brief Illness Perception Questionnaire thus evaluated is a reliable and valid tool.
Systemic lupus erythematosus and Still's disease are chronic autoimmune disorders of unknown etiology. Symptomatology of these diseases may be similar causing diagnostic difficulties. Long-term observation and immunological studies are essential to identify the definite disorder. We present a case of a 24-year-old patient with high fever, sore throat and arthritis. During hospitalization rash accompanying fever, nodular erythema, pulmonary changes, liver damage and splenomegaly were observed. Although initially adult-onset Still's disease was diagnosed according to the Yamaguchi criteria, the diagnosis of systemic lupus erythematosus was made after re-analysis of the clinical course and immunological tests.
