ASSESSMENT OF QUALITY OF LIFE IN PATIENTS WITH CHRONIC KIDNEY DISEASE AND PULMONARY HYPERTENSION: A PROSPECTIVE STUDY USING THE CAMPHOR QUESTIONNAIRE

Patients with chronic kidney disease (CKD) and pulmonary hypertension (PH) experience markedly impaired health-related quality of life (HRQoL), yet routine assessment using patient-reported outcome measures (PROMs) remains uncommon in clinical practice. The Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) questionnaire is a validated, disease-specific PROM widely used in Europe that captures three key domains: symptoms, activity limitations, and quality-of-life impact. While echocardiographic parameters provide objective measures of cardiac function, they may not fully reflect patients' functional capacity and symptom burden. Furthermore, the relationship between changes in pulmonary artery pressure and improvements in HRQoL during treatment remains poorly understood in the CKD population. This study aimed to assess HRQoL using CAMPHOR in CKD patients with PH and evaluate changes following 6 months of treatment with the endothelin-1 antagonist bosentan.

This prospective observational study enrolled 72 consecutive patients with CKD stages 3a–4 and echocardiographic evidence of PH from the Republican Scientific and Practical Center of Nephrology and Hemodialysis between January and December 2024. Inclusion criteria were: age ≥18 years, estimated glomerular filtration rate (eGFR) 15–59 mL/min/1.73m² (CKD stages 3a–4), and systolic pulmonary artery pressure (sPAP) ≥25 mmHg on transthoracic echocardiography. Exclusion criteria included dialysis dependence, acute kidney injury, severe valvular disease, or left ventricular ejection fraction <40%. All patients underwent comprehensive echocardiographic assessment including sPAP measurement by tricuspid regurgitation velocity and right ventricular function evaluation by tricuspid annular plane systolic excursion (TAPSE). The CAMPHOR questionnaire (25-item total score, range 0–50, higher scores indicating worse HRQoL) was administered at baseline and 6 months. Patients received bosentan 62.5 mg twice daily for 4 weeks, then 125 mg twice daily for 20 weeks, alongside standard CKD management. Renal function was monitored monthly. Statistical analysis used paired t-tests for within-group comparisons and ANOVA for between-group differences. The study was approved by the institutional ethics committee, and all participants provided written informed consent.

Data from 72 patients (41 males, 31 females; mean age 52 years, range 29–78) were analyzed: 24 with CKD stage 3a, 26 with stage 3b, and 22 with stage 4. At baseline, mean CAMPHOR total scores progressively worsened with advancing CKD stage: 19.3 (stage 3a), 25.5 (stage 3b), and 33.4 (stage 4), reflecting greater symptom burden and functional limitation in more severe kidney disease. Corresponding baseline sPAP values were 30.4, 35.8, and 41.3 mmHg, while TAPSE measured 21.0, 22.8, and 18.5 mm, respectively. Mean eGFR was 55.8 mL/min/1.73m² (stage 3a), 40.2 mL/min/1.73m² (stage 3b), and 26.1 mL/min/1.73m² (stage 4). After 6 months of bosentan therapy, CAMPHOR total scores improved significantly in all groups to 16.1, 21.4, and 27.9—representing reductions of 3.2 points (stage 3a, p=0.012), 4.1 points (stage 3b, p=0.008), and 5.5 points (stage 4, p=0.003). Notably, sPAP remained unchanged at follow-up (30.4, 35.8, and 41.3 mmHg). TAPSE showed modest, stage-specific changes: an increase of 1.8 mm in stage 3a (from 21.0 to 22.8 mm, p=0.042), a decrease of 1.8 mm in stage 3b (from 22.8 to 21.0 mm, p=0.035), and no change in stage 4 (18.5 mm, p=0.891). No significant decline in eGFR was observed during treatment (mean change -1.2 to -2.3 mL/min, p>0.05 for all stages). Treatment was well tolerated with no serious adverse events.

Six months of bosentan therapy in CKD stages 3a–4 with pulmonary hypertension yielded clinically meaningful improvements in health-related quality of life, as measured by CAMPHOR score reductions of 3.2–5.5 points across all CKD stages. Importantly, these symptomatic and functional gains occurred independently of changes in systolic pulmonary artery pressure, which remained stable throughout the treatment period. The modest and variable changes in TAPSE suggest that improvements in patient-reported outcomes may precede or occur without measurable echocardiographic improvements in right ventricular function. These findings underscore the importance of routine PROM assessment alongside echocardiography to comprehensively monitor treatment benefit in this population. The CAMPHOR questionnaire proved sensitive to detect clinically relevant changes in HRQoL and should be incorporated into standard care protocols for CKD patients with pulmonary hypertension. Further studies are needed to evaluate long-term outcomes and identify predictors of sustained quality-of-life improvement.