Clinical characteristics of Polish patients with ANCA-associated vasculitides-retrospective analysis of POLVAS registry.

OBJECTIVE: 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). METHODS: 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). RESULTS: 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). CONCLUSIONS: 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.

Illness perception is significantly determined by depression and anxiety in systemic lupus erythematosus.

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.

Association of HLA-DRB1 alleles with susceptibility to mixed connective tissue disease in Polish patients.

Mixed connective tissue disease (MCTD) is a systemic autoimmune disease, originally defined as a connective tissue inflammatory syndrome with overlapping features of systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), polymyositis/dermatomyositis (PM/DM) and systemic sclerosis (SSc), characterized by the presence of antibodies against components of the U1 small nuclear ribonucleoprotein (U1snRNP). The aim of the study was to assess the frequency of (high-resolution-typed) DRB1 alleles in a cohort of Polish patients with MCTD (n = 103). Identification of the variants potentially associated with risk and protection was carried out by comparison with the DKMS Polish Bone Marrow Donor Registry (41306 alleles). DRB1*15:01 (odds ratio (OR): 6.06; 95% confidence interval (CI) 4.55-8.06), DRB1*04 (OR: 3.69; 95% CI 2.69-5.01) and *09:01 (OR: 8.12; 95% CI 2.15-21.75) were identified as risk alleles for MCTD, while HLA-DRB1*07:01 allele was found to be protective (OR: 0.50; 95% CI 0.28-0.83). The carrier frequency of the DRB1*01 was higher in MCTD patients compared with controls, although the differences were not statistically significant. Our results confirm the modulating influence of HLA-DRB1 genotypes on development of connective tissue diseases such as MCTD.

Prevalence and correlates of suicidal thoughts in patients with neuropsychiatric lupus.

BACKGROUND: Suicidal ideation is observed in patients with systemic lupus erythematosus (SLE). No study on this notable phenomenon in neuropsychiatric SLE (NPSLE) is available so far. METHODS: Participants were 53 consecutive outpatients with NPSLE (48 women; mean age 43.8 years) diagnosed according to the American College of Rheumatology nomenclature for SLE neuropsychiatric syndromes. A Neuropsychiatric Questionnaire (NP-Q) concerning 45 neurological, cognitive and psychiatric symptoms was used to assess the prevalence of self-perceived neuropsychiatric symptoms. The Modified Hospital Anxiety and Depression Scale (HADS-M) was used to assess the level of anxiety, depression and irritability. Formal neuropsychological examination was performed. Clinical data were collected by means of medical charts review and structured interview. RESULTS: Suicidal thoughts were present in 25% of patients with NPSLE, irrespective of sex, age, education, work status, disease duration and steroid treatment. Suicidal ideation was connected with elevated levels of depression, anxiety and irritability. In patients with suicidal ideation the prevalence of cognitive, psychiatric and neurological self-perceived problems was significantly higher. CONCLUSIONS: Suicidal thoughts are common in patients with NPSLE. Neuropsychiatric manifestation per se, depression, anxiety and patients' subjective complaints can be risk factors for suicidal ideation. Screening for suicidal thoughts is vital in routine care of SLE patients.

Neuropsychological assessment in mixed connective tissue disease: comparison with systemic lupus erythematosus.

OBJECTIVE: The aims of the study were to assess cognitive functions (CF) in patients with mixed connective tissue disease (MCTD) and to compare MCTD patients with systemic lupus erythematosus patients with and without neuropsychiatric manifestations (NP-SLE and non-NP-SLE, respectively) in terms of CF. METHODS: Neuropsychological examination was performed in 141 patients: 30 with MCTD (24 women, 6 men), mean age: 48.07 years, 37 with non-NP-SLE (36 women, 1 man), mean age: 40.76 years and 74 with NP-SLE (68 women, 6 men), mean age: 41.97 years. Neuropsychological tests and structured interview were used. Emotional state was assessed by Hospital Anxiety and Depression Scale and clinical review. RESULTS: We observed cognitive impairment in six MCTD patients (20%); in one (3%) the impairment was severe. MCTD patients achieved significantly higher results in seven out of 11 tests compared with patients with NP-SLE. MCTD and non-NP-SLE patients did not differ significantly. The differences were irrespective of premorbid IQ, education, disease duration and steroid treatment. CONCLUSIONS: In the majority of MCTD patients, CF were not impaired and severe impairment was unusual. Cognitive functioning was most disturbed in NP-SLE. The cognitive deficits observed in connective tissue diseases can be connected with nervous system involvement.

Neuropsychological assessment in systemic lupus erythematosus patients: clinical usefulness of first-choice diagnostic tests in detecting cognitive impairment and preliminary diagnosis of neuropsychiatric lupus.

OBJECTIVES: To evaluate the usefulness of neuropsychological tests in order to distinguish the first-choice methods useful in quick detection of cognitive impairment in SLE and preliminary diagnosis of neuropsychiatric manifestation. Study aimed at assessment of the prevalence and severity of cognitive deficits in SLE patients and comparison between SLE patients with neuropsychiatric manifestations (NP-SLE) and without ones (non-NP-SLE). METHODS: 93 out of 104 SLE patients, 57 with NP-SLE and 36 with non-NP-SLE underwent comprehensive neuropsychological examination. Tailor-made structured interview for neuropsychological assessment in SLE (SISLE) was used. Patients' emotional state was assessed by clinical interview and HADS. RESULTS: Cognitive dysfunction was identified in 57% of SLE patients, 48.4% in 1-3 tests, 8.6% (8 patients) in 4 or more tests (severe decline). Among impaired patients 15% had severe decline. In NP-SLE group 63.2% were impaired vs. 47.2% in non-NP-SLE group. All 8 patients with severe decline were NP-SLE. The dysfunction was irrespective of premorbid intellectual level, age, education, disease duration and steroid treatment. In NP-SLE significantly lower scores were observed in 8 tests (10 parameters). CONCLUSIONS: Cognitive dysfunction is frequent in SLE patients. The majority of patients has mild deficits, but severe decline is also observed. The dysfunction is more frequent and more pronounced in NP-SLE. The study distinguished 8-test-first-choicebattery useful in detecting cognitive impairment in SLE and in case of severe decline - in preliminary differentiating NP-SLE and non-NP-SLE. Structured interview for psychological/neuropsychological examination of SLE patients is a useful and required tool for a standard patients' assessment.

[Clinical symptoms variety as a cause of late diagnosed Takayasu's arteritis]. / Róznorodnosc objawów klinicznych przyczyna póznego rozpoznania choroby Takayashu.

Takayasu's arteritis (TA) is a chronic inflammatory arteritis, preferentially affecting the major arteries such as the aorta and its main branches. Its course may be biphasic, with an early systemic phase characterised by non-specific inflammatory features and a later stage with occlusive lesion of the affected vessels. We reported 3 patients with TA, who presented very varied symptoms. These cases emphasise the importance of ultrasound image, which can help to establish the diagnosis at very early stage of vasculitis and improve prognosis.

A case of multisymptomatic relapsing polychondritis in a 22-year-old woman.

A case of multisymptomatic relapsing polychondritis in a 22-year-old woman. We report a case of a 22-year-old woman with relapsing polychondritis (RP)--a rare and little known systemic autoimmune disease characterised by episodic inflammation of cartilaginous structures (ear, nose, bronchi, trachea, larynx, ribs, cardiovascular system). This patient presents with a seven-year history, initiated by the saddle nose. The patient developed a multitude of symptoms: auricular chondritis, ocular symptoms, recurrent arthritis, respiratory complications (laryngotracheomalacia, bilateral vocal cord palsy), sensorineural hearing loss and enchondroma of the humeral bone. The examination of an auricle biopsy by an immunofluorescent method and a positive serum reaction from the patient to normal cartilage supported the immunological nature of relapsing polychondritis. Treatment consisted of orally administered prednisone and diaminodiphenylsulfone (Dapsone).