Referring Patients for PAH-CTD Diagnosis

Co-management of Patients With PAH-CTD

Rheumatologists are integral to the detection and co-management of PAH associated with systemic sclerosis (PAH-SSc). The identification, diagnosis, and management of PAH-SSc requires collaboration among pulmonologists, cardiologists, and rheumatologists.1,2 Given the complex nature of PAH and its diagnosis and management, it is important to consider partnering with specialists who have experience in managing this condition.2-4

PARTNERSHIP of PAH-SSc specialists
PAH-CTD medical spcialists flow chart
African American patients with PAH-SSc may be diagnosed with more severe disease.5 Find Out How They May Be at Greater Risk >

Referral

Utilizing the Pulmonary Hypertension Association’s network of Centers of Comprehensive Care and Regional Clinical Programs to collaborate with appropriate specialists may reduce PAH-SSc diagnosis delay and improve outcomes.6

Confirming a PAH diagnosis7

  • An RHC is required to confirm a diagnosis
  • The 2022 ESC/ERS Guidelines recommend that an RHC be performed in a PH center
The 2022 ESC/ERS Guidelines recommend patients with PAH receive multidisciplinary management with PAH specialists.7

Treatment Pathways in PAH

Early diagnosis and treatment may improve long-term outcomes in PAH7-9

  • The goal of PAH treatment is to achieve and maintain a low-risk status7
  • A risk assessment should be performed at diagnosis and follow-up7
    • A low-risk status at diagnosis focuses on WHO FC I or II, 6MWD >440 m, BNP <50 ng/L, NT-proBNP <300 ng/L, RAP <8 mmHg, CI ≥2.5 L/min/m2, SVI >38 mL/m2, and SvO2 >65%*
    • A low-risk status at follow-up includes WHO FC I or II, 6MWD >440 m, BNP <50 ng/L, or NT-proBNP <300 ng/L

Guidelines recognize that focusing on multiple pathways is an effective treatment strategy7

  • 3 separate signaling pathways in PAH can be targeted by different drug classes7,10:
Nitric Oxide Pathway (PDE-5is and sGC stimulators)7,10
Endothelin Pathway (ERAs)7,10
Prostacyclin Pathway (Prostacyclin pathway agents)7,10
Pathways Diagram Pathways Diagram
Focusing on multiple pathways has been proven to have clinical benefits, depending on
disease progression7,9
*Guidelines recommend taking into account as many factors as possible, but emphasize these parameters. The full list can be found in the Comprehensive Risk Assessment in PAH table (Table 16 in the 2022 ESC/ERS Guidelines).7
6MWD=6-minute walk distance; BNP=brain natriuretic peptide; CI=cardiac index; ERA=endothelin receptor antagonist; ERS=European Respiratory Society; ESC=European Society of Cardiology; FC=Functional Class; NT-proBNP=N-terminal pro-brain natriuretic peptide; PAH=pulmonary arterial hypertension; PAH-CTD=pulmonary arterial hypertension associated with CTD; PAH-SSc=pulmonary arterial hypertension associated with SSc; PDE-5i=phosphodiesterase type 5 inhibitor; PH=pulmonary hypertension; RAP=right atrial pressure; RHC=right heart catheterization; sGC=soluble guanylate cyclase; SVI=stroke volume index; SvO2=mixed venous oxygen saturation; WHO=World Health Organization.
References: 1. McLaughlin VV, Shah SJ, Souza R, Humbert M. Management of pulmonary arterial hypertension. J Am Coll Cardiol. 2015;65(18):1976-1997. 2. Minai OA, Budev MM. Diagnostic strategies for suspected pulmonary arterial hypertension: a primer for the internist. Cleve Clin J Med. 2007;74(10):737-747. 3. Strange G, Fowler R, Jary C, et al. Integrated care and optimal management of pulmonary arterial hypertension. J Multidiscip Healthc. 2009;2:67-78. 4. Deaño RC, Glassner-Kolmin C, Rubenfire M, et al. Referral of patients with pulmonary hypertension diagnoses to tertiary pulmonary hypertension centers: the multicenter RePHerral study. JAMA Intern Med. 2013;173(10):887-893. 5. Blanco I, Mathai S, Shafiq M, et al. Severity of systemic sclerosis-associated pulmonary arterial hypertension in African Americans. Medicine (Baltimore). 2014;93(5):177-185. 6. Mandras SA, Ventura HO, Corris PA. Breaking down the barriers: why the delay in referral for pulmonary arterial hypertension? Ochsner J. 2016;16(3):257-262. 7. Humbert M, Kovacs G, Hoeper MM, et al; ESC/ERS Scientific Document Group. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: the Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS). Eur Heart J. 2022;43(38):3618-3731. 8. Humbert M, Coghlan JG, Khanna D. Early detection and management of pulmonary arterial hypertension. Eur Respir Rev. 2012;21(126):306-312 9. Galiè N, Channick RN, Frantz RP, et al. Risk stratification and medical therapy of pulmonary arterial hypertension. Eur Respir J. 2019;53(1):1801889. doi:10.1183/13993003.01889-2018 10. Lang MI, Gaine SP. Recent advances in targeting the prostacyclin pathway in pulmonary arterial hypertension. Eur Respir Rev. 2015;24(138):630-641.