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Cardiology / Cardiovascular

PAH - Scleroderma Associated

ICD-10 Code
I27.21

Comprehensive clinical criteria for PAH - Scleroderma Associated

Clinical Presentation & Protocol

Patient Usually Complains Of

Patient presents for follow-up of Scleroderma-associated Pulmonary Arterial Hypertension (SSc-PAH). Reports [stable/worsening] dyspnea on exertion (NYHA Class [I-IV]), fatigue, and [presence/absence] of syncope or presyncope. Review of systems positive for Raynaud’s phenomenon, digital ulcers, and GERD. No reported chest pain or palpitations. Current medication adherence is [good/poor].

Clinical Examination Findings

Vitals: BP [X/X], HR [X], O2 sat [X]% on [RA/Liters O2]. General: Patient appears [non-distressed/chronically ill]. CV: Regular rate and rhythm, prominent P2, [presence/absence] of holosystolic murmur at left sternal border (tricuspid regurgitation), JVD noted at [X] cm. Pulmonary: Clear to auscultation bilaterally. Extremities: [Presence/absence] of pitting edema, skin shows sclerodactyly and telangiectasias.

Treatment Protocol

Plan: Continue current PAH-targeted therapy: [PDE5 inhibitor/Endothelin Receptor Antagonist/Prostacyclin analog]. Monitor LFTs, CBC, and renal function. Titrate dose as tolerated. Optimize management of underlying Scleroderma with [Immunosuppressants/PPIs/CCBs]. Schedule 6-minute walk test (6MWT) and repeat TTE/RHC as indicated to assess hemodynamic response.

Detailed clinical guide coming soon.