ABSTRACT
The UK Scleroderma Study Group developed guidelines on the diagnosis and management of scleroderma renal crisis (SRC) based on best available evidence and clinical experience. SRC is characterised by the acute onset of severe hypertension and acute kidney injury. Current strategies to reduce the associated morbidity and mortality include identifying at risk patients to aid early diagnosis. ACE inhibitor therapy should be lifelong in all patients, regardless of whether they require renal replacement therapy. Patients with SRC may recover renal function up to 3 years after the crisis, most often within 12 to 18 months.
Subject(s)
Acute Kidney Injury/diagnosis , Acute Kidney Injury/therapy , Hypertension/diagnosis , Hypertension/therapy , Nephrology/standards , Renal Replacement Therapy/standards , Rheumatology/standards , Scleroderma, Systemic/complications , Acute Kidney Injury/etiology , Acute Kidney Injury/mortality , Angiotensin-Converting Enzyme Inhibitors/administration & dosage , Anti-Arrhythmia Agents/administration & dosage , Anticonvulsants/administration & dosage , Antihypertensive Agents/administration & dosage , Consensus , Critical Pathways/standards , Drug Administration Schedule , Evidence-Based Medicine/standards , Humans , Hypertension/etiology , Hypertension/mortality , Predictive Value of Tests , Risk Factors , Scleroderma, Systemic/diagnosis , Scleroderma, Systemic/mortality , Scleroderma, Systemic/therapy , Treatment Outcome , United KingdomABSTRACT
OBJECTIVE: Pulmonary arterial hypertension (PAH) is a severe complication of connective tissue diseases (CTDs). This study aimed to investigate the clinical and hemodynamic characteristics and survival of anti-U1 RNP-positive patients with CTD-associated PAH, with a focus on systemic sclerosis (SSc)-associated PAH. METHODS: We implemented a prospective database that included patients with CTD-associated PAH for whom there were clinical, autoantibody, and mortality data. We compared clinical and hemodynamic characteristics to anti-U1 RNP antibody status. We then assessed whether anti-U1 RNP antibodies could be a prognostic factor in CTD-associated PAH with a focus on SSc-associated PAH. RESULTS: We studied a total of 342 patients with CTD-associated PAH, of whom 36 (11%) were anti-U1 RNP antibody positive. Anti-U1 RNP-positive patients were younger and less functionally impaired than were anti-U1 RNP-negative patients in CTD- and SSc-associated PAH. Hemodynamic parameters were similar in anti-U1 RNP-positive and anti-U1 RNP-negative patients. In CTD-associated PAH, anti-U1 RNP positivity was associated with decreased mortality in univariable analysis (hazard ratio 0.34 [95% confidence interval 0.18-0.65], P < 0.001). In multivariable analysis, anti-U1 RNP positivity was also associated with decreased mortality (hazard ratio 0.44 [95% confidence interval 0.20-0.97], P = 0.043) independently of age, sex, functional parameters, lung involvement, and hemodynamic parameters. Results were similar in SSc-associated PAH, although the association between anti-U1 RNP positivity and survival did not reach significance in univariable (hazard ratio 0.47 [95% confidence interval 0.22-1.02], P = 0.055) and multivariable (hazard ratio 0.47 [95% confidence interval 0.20-1.11], P = 0.085) analyses. CONCLUSION: Anti-U1 RNP positivity was associated with distinct clinical characteristics and survival in CTD- and SSc-associated PAH. While hemodynamic parameters were similar in anti-U1 RNP-positive and anti-U1 RNP-negative patients, our results suggest that anti-U1 RNP positivity could be a factor protecting against mortality in CTD- and SSc-associated PAH.
Subject(s)
Autoantibodies/immunology , Hypertension, Pulmonary/immunology , Ribonucleoprotein, U1 Small Nuclear/immunology , Scleroderma, Systemic/immunology , Adult , Age Distribution , Age Factors , Aged , Cohort Studies , Connective Tissue Diseases/complications , Connective Tissue Diseases/immunology , Connective Tissue Diseases/mortality , Databases, Factual , Female , Humans , Hypertension, Pulmonary/etiology , Hypertension, Pulmonary/mortality , Kaplan-Meier Estimate , Male , Middle Aged , Prognosis , Proportional Hazards Models , Prospective Studies , Scleroderma, Systemic/complications , Scleroderma, Systemic/mortality , Severity of Illness Index , Sex FactorsABSTRACT
INTRODUCTION: Since remission is now possible in psoriatic arthritis (PsA) we wished to examine remission rates in PsA patients following anti tumour necrosis factor alpha (TNFalpha) therapy and to examine possible predictors of response. METHODS: Analysis of a prospective patient cohort attending a biologic clinic, between November 2004 and March 2008, was performed prior to commencing therapy and at regular intervals. Baseline clinical characteristics including demographics, previous disease-modifying antirheumatic drug (DMARD) response, tender and swollen joint counts, early morning stiffness, pain visual analogue score, patient global assessment, C reactive protein (CRP) and health assessment questionnaire (HAQ) were collected. RESULTS: A total of 473 patients (152 PsA; 321 rheumatoid arthritis (RA)) were analyzed. At 12 months remission, defined according to the disease activity score using 28 joint count and CRP (DAS28-CRP), was achieved in 58% of PsA patients compared to 44% of RA patients, significant improvement in outcome measures were noted in both groups (P<0.05). Analysis of a subgroup of PsA and RA patients matched for DAS28-CRP at baseline also showed higher numbers of PsA patients achieving remission. Linear regression analysis identified the HAQ at baseline as the best predictor of remission in PsA patients (P<0.001). CONCLUSIONS: DAS28 remission is possible in PsA patients at one year following anti-TNF therapy, at higher rates than in RA patients and is predicted by baseline HAQ.
