Electrocardiographic detection of pulmonary hypertension in patients with systemic sclerosis using the ventricular gradient.

BACKGROUND: Pulmonary hypertension (PH) is a leading cause of death in systemic sclerosis (SSc) patients. The current study assessed the ability of the ECG-derived ventricular gradient (VG-RVPO) to detect PH and predict all-cause mortality in PH patients with subtypes of SSc differing in the extent of multi-organ involvement. METHODS: ECGs were obtained from 196 patients with limited and 77 patients with diffuse SSc included from our screening programme on cardiac complications. The association of the VG-RVPO with (1) the presence of PH, (2) conventional screening parameters and (3) survival in PH patients was assessed. RESULTS: In limited SSc patients an elevated VG-RVPO corresponded with the presence of PH (-5±12 mV.ms vs -22±16 mV.ms, P<0.01), correlated significantly with conventional screening parameters and had a better diagnostic performance than the presence of a right heart axis (AUC 0.81 vs 0.60; P=0.04). These differences were not observed in patients with diffuse SSc. An elevated VG-RVPO was associated with decreased survival in all SSc patients with PH (3 year survival 30% vs 64%, P=0.02). CONCLUSION: An elevated VG-RVPO is associated with PH in limited SSc patients and with decreased survival in all SSc patients with PH.

The global peripheral chemoreflex drive in patients with systemic sclerosis: a rebreathing and exercise study.

BACKGROUND: Exercise intolerance (EI) in systemic sclerosis (SSc) is difficult to manage by the clinician. The peripheral chemoreflex drive compensates for metabolic acidosis during exercise and may be related to EI. AIM: To assess the global peripheral chemoreflex drive (GPCD) in patients with SSc at rest and during exercise. METHODS: Consecutively tested SSc patients (n = 49) were evaluated by pulmonary function tests, carbon dioxide (CO2) rebreathing studies and non-invasive cardiopulmonary exercise testing (CPET). Results of their CO2 rebreathing tests were compared with those of controls (n = 32). Respiratory compensation for metabolic acidosis during CPET was defined by the occurrence of a sharp increase in minute ventilation (VdotE) and the ventilatory equivalent for CO2 (V'E and V'CO2) at the end of the isocapnic buffer phase. Euoxic (eVHR) and hyperoxic (hVHR) ventilatory responses to hypercapnia were measured and its difference (eVHR - hVHR) was considered to reflect the GPCD. RESULTS: In 45 patients with SSc, CPET results showed respiratory compensation at the occurrence of metabolic acidosis. eVHR - hVHR in patients with diffuse cutaneous SSc (dcSSc) differed significantly from that in patients with limited cutaneous SSc (lcSSc) and from that in controls (0.47 ± 0.38 (dcSSc) vs. 0.90 ± 0.77 (lcSSc) and 0.90 ± 0.49 (controls) l/min/mmHg; P = 0.04 and P = 0.03, respectively). CONCLUSIONS: Respiratory compensation for metabolic acidosis occurred in all patients. However, the GPCD was diminished in dcSSc patients, suggesting an altered control of breathing. Its assessment may help the clinician to better understand reported EI and exertional dyspnea in dcSSc patients.

Translation, cross-cultural adaptation, and validation of the UCLA Scleroderma Clinical Trial Consortium Gastrointestinal Tract Instrument (SCTC GIT) 2.0 into Dutch.

OBJECTIVES: To translate and adapt the University of California, Los Angeles Scleroderma Clinical Trial Consortium Gastrointestinal Tract Instrument 2.0 (UCLA SCTC GIT 2.0) into Dutch and validate it among Dutch systemic sclerosis (SSc) patients. METHODS: First, the UCLA SCTC GIT 2.0 questionnaire was translated and adapted according to international guidelines. The resulting Dutch GIT 2.0 was, in combination with the SSc Health Assessment Questionnaire (SHAQ) and Short Form-36 (SF-36) administered to SSc patients participating in a standardised medical assessment. Moreover, all previous clinical examinations and confirmed medical diagnoses related to GIT were extracted from the medical records. Internal consistency was determined by calculating Cronbach's alpha. To determine the reliability, the questionnaire was re-administered with an interval of two weeks to a subgroup of patients and the intraclass-correlation coefficient (ICC) was computed. Spearman correlation coefficients between GIT scores, SF-36 and SHAQ were computed. GIT scores were compared among patients with and without previous gastrointestinal examinations and/or diagnoses. RESULTS: Eighty-nine patients with a mean age of 53.6 (SD 12) years, and predominantly female (76%) were included. The median total GIT score was 0.17 (Cronbach's alpha 0.921). The test-retest reliability of the total GIT score was good (n=27; ICC 0.749). Overall, the GIT total scores correlated significantly with the SHAQ visual analogue scale intestinal complaints and the SF-36. Significant differences between GIT total and subscale scores of patients with and without previous gastrointestinal examinations and diagnoses were present. CONCLUSIONS: The Dutch GIT 2.0 questionnaire showed good internal consistency, construct validity and test-retest reliability.

Detection of pulmonary vasculopathy by novel analysis of oxygen uptake in patients with systemic sclerosis: association with pulmonary arterial pressures.

OBJECTIVES: During cardiopulmonary exercise testing (CPET) compromised pulmonary vasculature in patients with systemic sclerosis (SSc) may lead to increases in pulmonary arterial pressures (PAP) and decreased oxygen uptake. We hypothesised that this may lead into a disproportional heart rate (HR) increase with a corresponding V'O2/HR breakpoint and relates to systolic PAP at rest. METHODS: In a prospective design we evaluated V'O2/HR slopes for breakpoints. To understand its physiological meaning, we evaluated V'O2/HR and V'O2/mPAP slopes for breakpoints in a historic data set of SSc patients, in which CPET and right heart catheterisation was performed simultaneously. V'O2/HR slopes with a peak oxygen uptake outside the normal range were defined as pathologic. RESULTS: A breakpoint occurred in both V'O2/mPAP and V'O2/HR slope in 16/34 patients in the historic dataset and occurred in the V'O2/mPAP slope at a lower V'O2in 15 patients. In the prospective dataset, 73/121 patients showed a V'O2/HR breakpoint and achieved a significantly lower peak oxygen uptake compared to 48/121 patients without a V'O2/HR breakpoint (p=0.036). Mean systolic PAP in 41/121 patients with a pathologic V'O2/HR slope differed significantly from patients without a pathologic V'O2/HR slope (p=0.027). In 27/121 patients with a systolic PAP < 35 mmHg a pathologic V'O2/HR slope was observed. CONCLUSIONS: SSc patients with a V'O2/HR breakpoint are characterised by a decreased oxygen uptake, likely caused by sudden PAP increases during exercise. Importantly, in patients with normal resting SPAP pathologic V'O2/HR slopes were observed. This suggests that these patients are at risk for developing pulmonary hypertension.

Health care in systemic lupus erythematosus (SLE): the patient's perspective.

In order to provide more patient-centered care for patients suffering from systemic lupus erythematosus (SLE), we studied their current satisfaction and preferences regarding future health care delivery. We sent questionnaires to all SLE patients visiting the rheumatology outpatient clinic in Leiden, the Netherlands. The questionnaire comprised three topics: (a) health care needs using a modified version of SLE Needs Questionnaire (range 0-38), (b) satisfaction with care per provider (visual analogue scale, range 0 (not at all)-100 (very satisfied)), and (c) preferences for future healthcare (four items). One hundred and two patients (63 % response) reported an average of 16 (±6) health care needs, with all patients reporting a need in the physical domain. More needs were significantly associated with worse physical functioning and a higher educational level. The average satisfaction score was 73 (±19) with a lower overall satisfaction score being associated with younger age and an educational level higher or lower than average. Regarding preferences for future health care delivery, 75 % of patients showed interest in a yearly standardized medical assessment, 57 % in regular, specialized nurse contacts using internet, 50 % in a yearly inventory on the need for self-management support, and 36 % in an education course. The association of age, education level and physical functioning with health care needs, and/or satisfaction suggest that the delivery of care should be better tailored to the needs of subgroups of patients.

Translation, cross-cultural adaptation, and validation of the Mouth Handicap in Systemic Sclerosis questionnaire (MHISS) into the Dutch language.

The Mouth Handicap in Systemic Sclerosis (MHISS) is a French-generic questionnaire evaluating mouth-opening restriction, dryness, and esthetic concerns. The aim of this study was to translate and adapt the MHISS questionnaire into the Dutch language and evaluate its psychometric properties. The MHISS was translated according to international guidelines, field-tested among 16 systemic sclerosis (SSc) patients, and adapted. Subsequently, the Dutch MHISS was administered to 52 SSc patients visiting the outpatient or day patient clinic of a university hospital and readministered after 2 weeks. Internal consistency was tested by computing Cronbach's alpha. Test-retest reliability was determined by computing the intraclass correlation coefficient (ICC) and validity by determining associations with measures of overall functioning (Health Assessment Questionnaire (HAQ)), maximum mouth opening (MMO, in millimeter), subjective xerostomia (visual analog scale), and objective xerostomia (Saxon test). Patients had mean ± standard deviation (SD) age and disease duration of 55 ± 21 and 7.2 ± 7.3 years. Twenty-seven (52 %) patients had diffuse cutaneous SSc. The mean Dutch MHISS score was 17.5 (SD 10.0) with Cronbach's alpha being 0.862. Dutch MHISS scores differed significantly between patients with high and low disability levels (HAQ, MMO, and subjective and objective xerostomia divided according to the median; paired t test). Spearman rank correlations with HAQ (r = 0.599, p = 0.000), MMO (r = -0.518, p = 0.000), and subjective xerostomia (r = 0.536, p = 0.000) were moderate; correlation with objective xerostomia did not reach statistical significance. The ICC was 0.94. The Dutch version of the MHISS demonstrated good psychometric properties and is useful in assessing mouth disability in SSc patients.

Health care usage in Dutch systemic lupus erythematosus patients.

As a first step in the improvement of the organization of care for patients with systemic lupus erythematosus (SLE) we studied their health care usage and its determinants. A questionnaire was sent to 161 outpatients of the rheumatology clinic of a Dutch university hospital. The questionnaire comprised questions on health care usage, quality of life and sociodemographic characteristics. Disease characteristics were extracted from the medical record. Among the 102 responders (63% response rate) the proportions of patients reporting contacts with a rheumatologist because of SLE since onset of the disease and over the past 12 months were 100% and 83%, respectively. These proportions were 93% and 68% for all other medical specialists, 88% and 44% for the general practitioner, 78% and 44% for any health professional, 29% and 9% for care at home, 48% and 17% for hospital admissions and 29% and 2% for day-patient care. Younger age, major organ involvement, the use of immunosuppressants and worse physical functioning were found to be significantly associated with greater health care use. This study demonstrated that health care usage by SLE patients is substantial and involves a variety of health care services. Further research should be directed at patients' satisfaction and patients' needs regarding the optimal organization of integrated, multidisciplinary services that are accessible for SLE patients of all ages.

Randomized comparison of a multidisciplinary team care program with usual care in patients with systemic sclerosis.

OBJECTIVE: To compare the effectiveness of a multidisciplinary team care program with usual outpatient care in patients with systemic sclerosis (SSc; scleroderma). METHODS: We performed a randomized controlled trial comparing a 12-week multidisciplinary team care program (1 day per week; individual treatments, group exercises, and group education) with outpatient clinic care. Outcome measures included the Hand Mobility in Scleroderma (HAMIS) test, grip strength, maximal mouth opening (MMO), 6-minute walk distance (6MWD), maximum aerobic capacity (VO(2max) ), Checklist Individual Strength 20 (CIS-20), SSc Health Assessment Questionnaire (HAQ), and Short Form 36 (SF-36), assessed at 0, 12, and 24 weeks. Statistical comparisons of change scores were done by analysis of covariance. RESULTS: Twenty-eight patients were assigned to the intervention group (mean age 53.9 years, 15 of 28 with diffuse SSc) and 25 were assigned to the control group (mean age 51.7 years, 15 of 25 with diffuse SSc). Twenty-five patients (89%) in the intervention group completed the treatment program. At 12 weeks, there was a significantly greater improvement in grip strength (2.2 versus -1.8 kg; P = 0.001), MMO (1.4 versus -0.9 mm; P = 0.011), 6MWD (42.8 versus 3.9 meters; P = 0.021), and HAQ score (-0.18 versus 0.13; P = 0.025) in the intervention group, whereas differences for the other outcome measures did not reach significance. At 24 weeks, the effect on grip strength persisted. CONCLUSION: In patients with SSc, a 12-week multidisciplinary day patient treatment program was more effective than regular outpatient care with respect to 6MWD, grip strength, MMO, and HAQ score, but not for VO(2max) , HAMIS test, CIS-20, SF-36, and visual analog scale for pain. This study provides a first step in quantifying the effect of a multidisciplinary team care program and warrants the conduct of further intervention studies.

Impaired sexual function in women with systemic sclerosis: a cross-sectional study.

OBJECTIVE: To compare sexual functioning and distress in women with systemic sclerosis (SSc) with that in healthy controls and determine the association between disease characteristics and sexual function. METHODS: We conducted a cross-sectional study of 69 women with SSc (ages 18-60 years) and 58 healthy, age-matched controls. Assessment included the Female Sexual Function Index (FSFI), Female Sexual Distress Scale (FSDS), Hospital Anxiety and Depression Scale, Short Form 36 health survey, sociodemographic characteristics, and in patients only, the Health Assessment Questionnaire. RESULTS: Of 69 eligible patients with SSc, 37 (54%) responded, in addition to 37 (64%) of 58 controls. The FSFI total score and the subscale scores for lubrication, orgasm, arousal, and pain were significantly lower and the FSDS scores were significantly higher in patients with SSc. Longer disease duration and higher levels of marital dissatisfaction were significantly associated with low sexual function in patients with SSc. Longer disease duration, more depressive symptoms, and the use of antidepressants were significantly associated with sexual distress. Multivariate analyses indicated that marital distress was the only variable significantly associated with low sexual function in patients with SSc (beta = 0.40, P < 0.05), whereas depression was the only variable significantly associated with sexual distress (beta = 0.32, P < 0.05). The same pattern of associations was found in the healthy control group. CONCLUSION: Women with SSc reported significantly impaired sexual functioning and more sexual distress then healthy controls. Impaired sexual functioning and sexual distress were associated with marital distress and depressive symptoms. These results indicate that in daily practice, inquiring about sexuality and screening for depressive symptoms is indicated in every patient with SSc, irrespective of their clinical characteristics.

Embolization of a ruptured aneurysm in classic polyarteritis nodosa presenting as perirenal hematoma.

A 26-year-old man was admitted with abdominal pain, anemia, and hypertension. Ultrasonography and computed tomography revealed a perirenal hematoma. Soon after admission, the patient went into shock, and emergency angiography was performed. Two active bleeding sites were found in the left kidney and were successfully embolized. Also, multiple aneurysms, consistent with the diagnosis of classic polyarteritis nodosa, were seen. After treatment was initiated, the patient recovered fully. When a spontaneous perirenal hematoma is a presenting symptom of classic polyarteritis nodosa, a delay in making the right diagnosis is likely to occur. Angiography not only may provide a quick diagnosis, but it can also be used to perform therapeutic embolization. In patients with classic polyarteritis nodosa and hemorrhage from a ruptured aneurysm, angiography may be a therapeutic alternative to surgery.