| |
2008 Q2 - QUARTERLY UDPATE
James R. Seibold, M.D. - Chair
Richard M. Silver, M.D.
Virginia D. Steen, M.D
Daniel E. Furst, M.D. |
Vallerie V. McLaughlin, MD
Anatoly Langer MD
Nick Hill MD |
Pulmonary involvement in systemic sclerosis (SSc) has emerged as the leading cause of disease-related morbidity and mortality.
It is characterized by pulmonary arteriolar fibrosis leading to pulmonary arterial hypertension (PAH), interstitial lung disease (ILD) which usually manifests as non-specific interstitial pneumonia (NSIP) or an admixture of both processes.
Moderate to severe restrictive lung disease has been identified in up to 40% of patients.
Early reductions in FVC represent an important risk factor for the development of end-stage ILD with greatest rate of deterioration observed in the first four years.
McLaughlin VV. Chest. 2004;126:78S–92S.
Survival of patients diagnosed with PAH depends on the etiology of their disease, as well as potential underlying conditions, including HIV disease, which may impact on survival.
Reference:
McLaughlin VV, Presberg KW, Doyle RL, et al. Prognosis of pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest. 2004;126:78S-92S.
The unfavourable clinical outcome associated with pulmonary complications of SSc has fuelled an intense search for new therapeutic strategies and an emphasis on the early detection of pulmonary pathology.
At present, these risk factors are thought to include older age, limited skin disease and reduced diffusing capacity of carbon monoxide.
Critical questions remain:
| 1. |
is there a clearly definable scleroderma phenotype at high risk of development of pulmonary hypertension. |
| 2. |
does recognition of earlier stages of disease lead to improved outcomes by permitting earlier specific intervention. |
| 3. |
does the approach to scleroderma lung disease in the primary care setting differ from that of tertiary referral centers. |
| 4. |
can consensus be developed regarding elevated standards of care both in diagnosis and management of PAH in scleroderma. |
QuERI is a large scale, multi-disciplinary quality improvement research initiative designed to ensure excellence in scleroderma long-term care setting.
QuERI represents an innovative integration of guidelines and clinical care delivery, to identify and implement evidence based practices in routine health care setting.
| • |
Provides clear guidelines for patient management (evidence-based approach) |
| • |
Helps physicians optimize diagnostic and therapeutic approach to PAH |
| • |
Helps physicians to manage their patients according to guidelines (case-based intervention) using patient management algorithm |
| • |
Asks physicians to send information (case report form) on how they manage their patients |
| • |
Provides feedback to physicians in comparison to national and regional standings |
| • |
Patient follow-up (ethics approved) provides opportunity to assess benefit |
| • |
Provides database for future research |
| • |
To facilitate: |
| |
- |
(1) access to best practice guidelines/suggestions in diagnosis of PAH and |
| |
- |
(2) adherence to the guidelines as part of patient management. |
| • |
The electronic case report form will provide a platform for diagnostic and therapeutic work up of scleroderma patients for possible PAH. |
| • |
Integration of this approach into physician's work environment will provide opportunities to apply the standard of care. |
| 1. |
To optimize patient care by providing patient care map for early PAH diagnosis and management through the process of data collection and database analysis. |
| 2. |
To further close the care gap in PAH diagnosis and management by providing feedback to individual physicians on their style compared to scleroderma experts and the national and regional averages. |
| 3. |
To follow scleroderma patients long-term (3 years) and to document outcomes in relation to natural history, baseline features, and treatment received. |
|
|
| 1. |
Identify best practice guidelines |
| 2. |
Define existing practice patterns and outcomes and current variations from best practice |
| 3. |
Identify and implement interventions to promote best practice |
| 4. |
Look for best practice to improve outcome and health-related quality of life |
| Step 1: |
Current guidelines are provided re: diagnostic work up after inclusion criteria are satisfied. |
| Step 2: |
|
| |
a. |
Practical steps towards confirming diagnosis: essential testing - all patients. |
| |
b. |
Contingent tests - selected patients. |
| Step 3: |
Collect data on current patient management while providing guidelines and evidence-based approach. |
| Step 4: |
Interactive continuing professional development as part of QuERI: implementation of learned principles into practice, i.e. enhancement of care and closure of care gap through physician feedback. |
| Inclusion Criteria: |
| 1. |
Patients > 18 years of age |
| 2. |
Scleroderma |
ACR criteria:
- Major
- minor |
| 3. |
Consent |
Exclusion Criteria: |
| 1. |
Unavailable for follow up |
| 2. |
Previously documented PH/PAH (right heart mPAP > 25 mm Hg, PVR > 3 Woods Units, PCWP < 15 mm Hg) |
| 3. |
Severe ILD (FVC < 45% predicted ) |
| 4. |
Overlap with other CTD (e.g. MCTD) |
Demographic data: including year of birth, gender, ethnic origin, year of 1st symptom of Raynauds, year of scleroderma diagnosis, SSc type, digital ulcer history, and history of pericarditis.
Organ Involvement: skin, GI, heart, lungs, skin, extremities.
Clinical information: NYHA classification and record symptoms only to include: presence of dyspnea, rales, digital tip ulcers, peripheral edema, and record skin involvement.
Medications: including but not limited to home O2, prednisone, cyclophosphamide, methotrexate, treatments for arrhythmias, and treatments for CHF, calcium channel blockers, ACE inhibitors, endothelin antagonists, prostacyclins, PDE-5 inhibitors other than prn use for sexual dysfunction.
Laboratory values: including auto-antibodies (ANA, ANA pattern, anti-centromere, anti-topoisomerase-1 (Scl-70)). Hemoglobin (Hb), Serum Creatinine (Cr), CPK, proNT-Brain natriuretic peptide (BNP).
Pulmonary function tests (PFTs): PFTs will be performed at the same lab at each yearly visit if possible. Standard calculations according to the American Thoracic Society will be used for percent-predicted forced vital capacity (FVC) and diffusing capacity for carbon monoxide (DLCO) and the FEV1 and TLC.
Echocardiograms: A written protocol and specific report form will be given to the echocardiographer (Appendix 3) in order to standardize reporting. Other parameters collected besides the estimated systolic PAP include tricuspid jet velocity, right heart abnormalities, the left ventricular (LV) ejection fraction, and diastolic dysfunction. Agitated saline infusion, which is the standard way to better demonstrate tricuspid regurgitation, will be used if necessary.
| RHC is recommended when: Dyspnea at NYHA Functional Class II or greater AND 2 out of the 4: |
| 1. |
Physical examination evidence of PH including increased P2, RV gallop, murmur of tricuspid regurgitation, evidence of right heart failure |
| 2. |
DLCO less than 55% of predicted or FVC/DLCO ratio > 1.4 |
| 3. |
Doppler echocardiogram with TR jet > 3.0 m.sec or estimated RVSP > 40mmHg |
| 4. |
proNT BNP > 200 pg/ml |
+ Click to enlarge
|
|
Scleroderma Steering Commitee
Needs Assessment Survey