q108 Q2 Q3 Q4 Q1 2009 Q2 2009 Q3 2009 Q4 2009
 
Welcome to the 2009 Q3 Scleroderma QuERI Quarterly Update,
Please select a category below or scroll down to read the newsletter.
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Introduction
Program Steps
Baseline Characteristics
Non Invasive Assesement

 


Echocardiography Results
Steering Committee Considerations
Summary


Needs Assessment Survey


 


This Update is intended to summarize the proceedings of the 2009 meeting for participants in the Scleroderma QuERI that took place at the ACR in Philadelphia.

QuERI is a Quality Enhancement Research Initiative that aims to improve management of patients with scleroderma who are at risk for developing pulmonary arterial hypertension (PAH), mostly among the community rheumatologists.

The steps of the QuERI can be summarized as follows:

•

Link guidelines and risk based management with actual patient care
• Monitor outcome and provide feedback to improve care
• Ensure dynamic (over a period of time) and interactive environment (allowing for changes)
• Use data for future research direction and guidelines development

The objectives of this initiative are:

•

To optimize patient care by providing patient care map for early PAH diagnosis and management through the process of data collection and database analysis.
• To 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.
• To follow scleroderma patients long-term (3 years) and to document outcomes in relation to natural history, baseline features, and treatment received.
• To measure and close hypothesized "care gap"



The steps of the program are simple, as shown below, and as of the September 2009 data cut there were 28 sites across the United States participating in the QuERI with 214 patients enrolled:

Step 1:
Identify scleroderma patients in community rheumatology practice suitable for enrollment.
Step 2:
Follow practical steps towards screening for and diagnosis of PAH.
Step 3:
Collect data on current patient management.
Step 4:

Collect data on long term outcome to establish incidence of PAH and related outcomes.

Patients included resembled those seen in clinical practice based on the criteria below:

Inclusion Criteria:

1.

Patients >= 18 years of age
2. Scleroderma by ACR criteria
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)
   




The following baseline characteristics have been determined to date:

SCLERODERMA QuERI (n=207)

Age (yrs) 57 (49,66)*
Female (%) 90.0%
Caucasian (%) 82.6%
Raynaud phenomenon 87.0%
Duration RP (yrs) 9 (4,15)*
Skin involvement 83.6%
GERD symptoms 59.9%
Digital ulcers 30.0%
Peripheral edema 17.7%
Rales 15.0%
*Median, 25th and 75th percentile


The physical exam disclosed the following:

SCLERODERMA QuERI (n=207)

BMI 25.1 (22.4, 29.4)*
Systolic BP 120 (110, 132)*
Diastolic BP 70 (66, 80)*
Heart Rate (bpm) 76 (68,83)*
Increased P2 5.8%
RV Gallop 2.4%
*Median, 25th and 75th percentile

The non-invasive assessment disclosed the following:

SCLERODERMA QuERI (n=207)
Pulmonary Function Tests

DLCO 15.8 (12.8, 18.8)*
DLCO % predicted <55 27.2%
FVC 2.7 (2.3, 3.2)*
FVC/DLCO > 1.4 44.9%
DLCO % predicted <55 and FVC/DLCO > 1.4 19.6%
*Median, 25th and 75th percentile

Next Table >> Other tests


 


SCLERODERMA QuERI
Other Tests

Hemoglobin (gm/dL) 12.7 (11.9, 13.8)*
Abnormal ANA 58.2%
BNP Done 23.8%
BNP Abnormal
(>140 pg/ml)** 		12.2%
6MWT 10.6%
Distance (m)** 410 (298, 479)*
*Median, 25th and 75th percentile **Proportion out of patients reported

The results of the echocardiography were as follows:

SCLERODERMA QuERI (n=176)
Echocardiogram

Tricuspid Regurg Reported 48.3%
Max Jet Velocity > 3.0 m/sec 11.1%
RVSP Reported 32.4%
RVSP > 40mm Hg* 20%
*Proportion out of patients with RVSP value reported

Because of the known issues with echocardiography as shown below

+ click to view larger



The steering committee also recommended the following considerations when deciding on the need for right heart catherization in patients with the possible diagnosis of PAH:

•

Echo estimated RVSP > 40 mm Hg or TR jet > 3.0 m/sec
• DLCO < 55% AND FVC/DLCO ratio > 1.4
• NTproBNP > 140 pg/ml
• Physical Exam - increased P2, RV gallop, murmur of TR, signs of RH failure
• Any two of four plus NYHA FC II dyspnea

The following is the summary of the use of right heart catheterization to date

SCLERODERMA QuERI
Use of Diagnostic Tests

Rec/Contingent
Dx Tests 		Community
(n=168)		 Academic
(n=39)		 p Value
RHC 5 (3.0%) 5 (12.8%) 0.02
+ CRITERIA FOR PAH ON RHC 4/5 0  

Overall use of the diagnostic tests differed as follows:

SCLERODERMA QuERI
Use of Diagnostic Tests

Rec/Contingent
Dx Tests 		Community
(n=168)		 Academic
(n=39)		 p Value
PFTs 79.2% 97.4% 0.007
Echo 83.9% 89.7% 0.36
VQ Lung Scan 1.2% 0% 1.0
ECG 10.1% 15.4% 0.40
6MWT 6.6% 28.2% <0.0001
HRCT Scan 29.8% 69.2% <0.0001
CXR or CT 59.5% 79.5% 0.02



In summary:

•

Most scleroderma patients are middle-aged or older women with only a mild respiratory disability.
• Diagnostic work up and reporting is not uniform.
• Practice setting appears to influence use of various diagnostic testing
• In the community RVSP is reported in only 32% of echocardiograms despite the widespread use of echocardiography for early detection of PAH in high-risk patients
• Certain diagnostic tests, e.g. RHC, are underutilized.
• More consistent approach to these high risk patients may optimize and improve their care and outcome.

 

Click here to complete a short
Needs Assessment Survey


these data were presented as an abstract at the ACR 2009
by Seibold et al