Recommendations For Screening and Detection Of Connective-Tissue Disease Associated Pulmonary Arterial Hypertension

Dinesh Khanna¹, Heather Gladue², John D. FitzGerald³, Richard N. Channick⁴, Lorinda Chung⁵, Oliver Distler⁶, Daniel Furst⁷, Eric Hachulla⁸, Marc Humbert^9,10,11, David Langleben^12,13, Stephen C. Mathai¹⁴, Rajeev Saggar¹⁵, Scott H. Visovatti¹⁶ and Vallerie McLaughlin¹⁷, ¹Division of Rheumatology, University of Michigan Medical Center, Ann Arbor, MI, ²University of Michigan, Ann Arbor, MI, ³Medicine/Rheumatology, UCLA School Med Rehab #32-59, Los Angeles, CA, ⁴Pulmonary Hypertension Program, Massachusetts General Hospital, Boston, MA, ⁵Rheumatology, Stanford Univ Medical Center, Palo Alto, CA, ⁶Division of Rheumatology, University Hospital Zurich, Zurich, Switzerland, ⁷Rheumatology, University of California in Los Angeles, Los Angeles, CA, ⁸Department of Internal Medicine, Claude Huriez University Hospital, Lille, France, ⁹AP-HP Hôpital Antoine Béclère, Clamart, France, ¹⁰Université Paris-Sud, Le Kremlin-Bicêtre, France, ¹¹NSERM U999, Centre Chirurgical Marie-Lannelongue, Le Plessis-Robinson, France, ¹²Cardiology Division, Jewish General Hospital, Montreal, QC, Canada, ¹³McGill University, Montreal, QC, Canada, ¹⁴Medicine, Johns Hopkins University, Baltimore, MD, ¹⁵Critical Care Medicine, St. Joseph's Hospital and Medical Center, Phoenix, AZ, ¹⁶Internal Medicine, Division of Cardiology, The University of Michigan, Ann Arbor, MI, ¹⁷Internal Medicine, Division of Cardiology, University of Michigan, Ann Arbor, MI

Meeting: 2013 ACR/ARHP Annual Meeting

Keywords: Systemic sclerosis

Session Information

Title: Systemic Sclerosis, Fibrosing Syndromes, and Raynaud’s - Clinical Aspects and Therapeutics II

Session Type: Abstract Submissions (ACR)

Background/Purpose: Pulmonary arterial hypertension (PAH) affects up to 15% of patients with connective tissue diseases (CTD) and is one of the leading causes of mortality in systemic sclerosis (SSc) and mixed connective tissue disease (MCTD). Previous recommendationswere developed as part of larger efforts in PAH and did not provide detailed recommendations in CTD-PAH. To develop recommendations for screening and early detection of CTD-PAH using rigorous data-driven and consensus-building methodology.

Methods: We performed a systematic review for the screening and diagnosis of PAH in CTD by searching available databases. Using the RAND/UCLA methodology, we developed case scenarios followed by 2 stages of voting—first one was voted anonymously by 10 international experts on 1 (inappropriate) -9 (appropriate) scale and 2^nd voting after face-to-face meeting.

Results: The key recommendations include:

1. All patients with SSc should be screened for PAH.

2. MCTD or other CTD’s with scleroderma features (referred hereon as scleroderma-spectrum disorders) should be screened similar to patients with SSc.

3. Screening of asymptomatic patients is not recommended for MCTD or other CTD patients without features of scleroderma.

4. RHC is mandatory for diagnosis of PAH.

5. Acute vasodilator testing is not required as part of the evaluation of PAH in patients with SSc, SSc-spectrum disorders, or other CTDs.

6. Initial screening evaluation in patients with SSc and scleroderma-spectrum disorders include pulmonary function test (PFT) including diffusion capacity carbon monoxide (DLCO), Transthoracic echocardiogram (TTE), NT- Pro BNP, and DETECT algorithm if DLCO% < 60% and >3 years disease duration.

7. In SSc and SSc-spectrum disorders, TTE and PFT should be performed on an annual basis or TTE, PFT, and NT-Pro BNP if new signs or symptoms develop.

Recommendations for RHC for SSc and scleroderma-spectrum disorders

Non-invasive test	Threshold for RHC	*Signs or symptoms required for RHC**
TTE	TR velocity of 2.5-2.8 m/s	Yes
	TR velocity of >2.8 m/s	No
	Right atrial ( RA major dimension >53 mm) or right ventricular enlargement ( Mid cavity RV dimension > 35 mm), irrespective of TR velocity	No
PFTs	FVC/DLCO ratio > 1.6 &/or DLCO <60%**	Yes
	FVC/DLCO ratio >1.6 &/or DLCO<60% & NT-Pro BNP >2 times upper limit of normal**	No
Composite measure	Meets DETECT algorithm in patients with DLCO< 60% & disease duration> 3 years	No
* Symptoms: dyspnea on rest or exercise, fatigue, pre-syncope/ syncope, chest pain, palpitations, dizziness, lightheadedness. Signs: Loud pulmonic sound, peripheral edema **TTE without overt systolic dysfunction, greater than grade I diastolic dysfunction or greater than mild mitral or aortic valve disease or evidence of PH

Conclusion: We provide consensus-based and evidence-driven recommendations for screening and early detection of CTD-PAH. It is our hope that these recommendations will lead to early detection of CTD-PAH and ultimately improve patient outcomes.

Disclosure:

D. Khanna,

NIH,

Scleroderma Foundation,

Actelion Pharmaceuticals US,

Gilead,

United Therapeutics,

Roche Pharmaceuticals,

BMS,

DIGNA,

Merck Pharmaceuticals,

H. Gladue,
None;

J. D. FitzGerald,
None;

R. N. Channick,

Actelion, United Therapeutics, Bayer,

Actelion, United Therapeutics,

L. Chung,
None;

O. Distler,

Sanofi, Active Biotech, Pfizer, Actelion, and Novartis,

Actelion, Sanofi-Aventis, Roche, Genentech, Active Biotec, medac, Novartis, Sinoxa, 4D Science,

D. Furst,

AbbVie, Actelion, Amgen, BMS, Gilead, GSK, NIH, Novartis, Pfizer, Roche/Genentech, UCB,

AbbVie, Actelion, Amgen, BMS, BiogenIdec, Janssen, Gilead, GSK, NIH, Novartis, Pfizer, Roche/Genentech, UCB,

AbbVie, Actelion, UCB,

E. Hachulla,

Actelion, GSK, Pfizer, Lilly, United Therapeutics,

M. Humbert,
None;

D. Langleben,
None;

S. C. Mathai,
None;

R. Saggar,

Gilead, Actelion, United Therapeutic,

S. H. Visovatti,
None;

V. McLaughlin,

Actelion, Bayer, Gilead, Merck, United Therapeutics.,

Gilead, United Therapeutics,

Actelion, Ikaria, Novartis, and United Therapeutics.,

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