BackgroundSystemic sclerosis (SSc) is a chronic, autoimmune disease of the connective tissue that involves skin, subcutaneous tissue, muscles and joints, as well as the internal organs: kidneys, lungs, heart. Depending on the extent it can occur as limited (lcSSc) or diffuse (dcSSc) clinical variant. About half of the patients with renal involvement the clinical manifestation is limited to a moderate increase in serum creatinine, mild proteinuria, and moderate hypertension. The most serious complication remains sclerodermal renal crisis (SRC). There was significant heterogeneity in definitions. As a rule for SRC was characterised by new-onset severe hypertension, hypertensive encephalopathy and seizures, acute kidney injury with oliguria, proteinuria and erythrocyturia, and microangiopathic hemolytic anaemia with thrombocytopenia.ObjectivesThe aim of our study was to analyse the clinical features and outcome of patients with scleroderma renal crisis.MethodsWe retrospectively reviewed the medical records of patients with scleroderma renal crisis between January 2002 and December 2016. Conditional logistic regression and multivariate analysis was performed to determine factors independently associated with outcome in patients with SRC.Results18 (14 females and 4 males, median age 47,3±14,6 years) patients diagnosed with SRC were included in the study. The mean duration from first symptom of systemic sclerosis (Raynaud’s phenomenon, cutaneous sclerosis, arthralgia/arthritis, puffy hands, interstitial lung disease, pulmonary arterial hypertension, digestive hypomotility and etc.) to SRC attack was 4,47±2,9 years. 13 SRC patients belonged to dcSSc, and 5 patients – to lcSSc. Among SRC patients, 9 were negative of anti-centromere antibodies (ACAs). All these 18 patients had hypertension and renal insufficiency, including 4 dialysis dependent after the onset of SRC and 6 with thrombotic microangiopathy. There were 6 patients receiving renal biopsy (the pathological findings were mainly summarised as intimal thickening and stenosis of renal arterioles). Among 16 patients with long-term followed-up, 15 patients received angiotensin converting enzyme inhibitors (ACEI), 8 patients died, 1 patient was dialysis dependent. Survival from first symptom in those patients with Raynaud’s phenomenon mode of onset was higher at 5 years and at 10 years than those with onset as non-Raynaud’s phenomenon (p < 0,05). In multivariate analysis, factors related to mortality in SRC were older age at onset, male gender, treatment with corticosteroids, dcSSc subset, interstitial lung disease, pulmonary arterial hypertension, heart involvement, and the mode of onset with non-Raynaud’s phenomenon, especially in the form of pulmonary involvement. The mode of onset should be considered an independent prognostic factor in SRS. The treatment of SRC relies on aggressive blood pressure control with an ACEI, combined with other antihypertensive drugs if needed.ConclusionsSRC usually occurred at the early course of SSc. dcSSc was more frequent than lcSSc....

BackgroundDermatomyositis (DM) and polymyositis (PM) are characterized by moderate to severe muscle weakness and inflammatory lesions in the muscle. They lead to frequent, and, in some cases, life-threatening extramuscular (lung, cardiac, neurologic, kidney) complications.ObjectivesThe aim of this study was to determine the incidence, the severity, the course and outcome of renal diseases in patients with DM or PM.MethodsWe identified 84 patients (68 female, 16 male) with the diagnosis of DM and 23 (14 female, 9 male) with PM. Diagnosis of DM and PM was based on the ENMC classification. The mean follow-up was 5,6 ± 3,8 years. Evaluation of extramuscular involvement was mainly performed in the presence of suggestive clinical signs. Autoantibodies were detected by ELISA. Acute kidney injury (AKI) was defined as an acute doubling of serum creatinine level. Chronic kidney disease (CKD) was defined if an estimated glomerular filtration rate (eGFR) <60 mL/min on at least 2 measurements 3 months apart.ResultsOf the 107 patients, 26 were found to have suffered varying degree of renal involvement. All the 26 patients (22 patients with DM and 4 with PM) had varying degree of proteinuria and haematuria. Renal involvement consisted of aKI in 8 patients and CKD in 18 patients. Among patients with aKI, 6 (75%) progressed to CKD, including 2 patients who reached end-stage renal disease (ESRD). Acute tubular necrosis with renal failure developed in 3 patients with PM and in 4 patients with DM. In descriptive analysis, male sex, cardiovascular risk factors, the severity of muscle injury, heart involvement, and initial proteinuria (>0.3 g/d) were associated with aKI. In multivariate survival analysis, only age at disease onset, male sex, history of cardiovascular events, and a previous episode of aKI were identified as risks factors of CKD. Although the proteinuria and haematuria responded to therapy (except in 2 cases), the creatinine clearance remained decreased during the treatment. Kidney biopsy was performed in 16 patients and showed chronic tubulointerstitial nephritis (7 cases), minimal change disease (2 cases), focal segmental glomerulosclerosis lesions (3 cases), IgA nephropathy (2 cases), membranous nephropathy (1 case), and amyloid deposits (1 case).ConclusionOur data clearly indicate that DM and PM are associated with a wide range of renal disorders. Renal damage was noted in 24,3% of all included patients. AKI occurred in 7,48% of cases and CKD developed in 16,82% of the patients. AKI and CKD contribute to an increased morbidity in these patients. AKI and CKD are probably not a direct manifestation of PM or DM, but rather result from drug and myoglobin-induced renal damage. However, aKI and CKD contribute to increased morbidity in these patientsDisclosure of interestsNone declared

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BackgroundRheumatoid arthritis (RA) is a chronic representative inflammatory autoimmune disease. The association of disease activity and pro-inflammatory cytokines with depression has not been sufficiently investigated.ObjectivesThe aim of this study is to analyze the association between disease activity and depression using Patient Help Questionnaire (PHQ-9) in patients with rheumatoid arthritis (RA). We also examined the outcome of intervention on depression score and determined the prevalence of depression and risk factors for depression and deterioration of depressive symptoms in RA patients.Methods146 RA patients with a mean age of 51,3±11,2 years were included in the study. Demographic and laboratory data were examined. Disease activity score 28-joint count C-reactive protein (DAS 28-CRP) was performed to assess disease activity of RA. PHQ-9 scores were collected at each clinic visit. Physicians assessed corresponding disease activity using Clinical Disease Activity Index (CDAI). Patients with at least moderate depression (PHQ-9 _10) were offered depression intervention, counseling or medications. PHQ-9 was re-administered after intervention.Results119 of RA patients were females, the average disease duration was 6,8 ± 5,9 years. Depression was diagnosed in 38 of RA patients: 18 - mild, 13 - moderate and 7 - moderately severe. Severity of depression positively correlated with disease activity in RA patients (p <0.05). RA patients with moderate/high CDAI had significantly higher PHQ-9 than those with low CDAI (p<0.001). Of 7 patients who met criteria for depression intervention, 6 were treated and 1 - declined. With treatment 5 patients had improved PHQ-9 scores, 1 patient worsened, and 1 patient had no change in score. The risk of developing a depressive disorder is highest between 5 and 10 years of onset of the disease and depression is a better predictor of work disability than disease activity and response to treatment. Depression is associated with more pain, fatigue and impaired quality of life. Therefore, the risk to develop a depression is increased with impaired function as measured by the health assessment questionnaire (HAQ). Increased disease activity increases the risk for depression in RA. The severity of disease activity of RA, DAS 28-CRP [OR 1.75, 95% CI 1.08-2.64]) and severity of fatigue (OR 1.32 95% CI 1.12-1.27) were associated with depression and deterioration of depressive symptoms in the multivariate analysis. Among the components of DAS 28-CRP, patient assessment for global health and abilities for daily performance were more related to depression. Depression unfavorably influences the response to therapy, the rate of remission is lower and the mortality is increased in RA patients. Taken together, this indicates that it is necessary to detect a depression in patients with RA as early as possible in order to initiate appropriate treatment of depression in such cases.ConclusionOur study shows depression in 19,18% of patients. Correlation between disease activity and depression score is ...