Michelle T
 | 47-year-old female |
| | Married with 2 children |
| | Employed (laboratory technician) |
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| | Weight: 181 lb |
| | Nonsmoker, rare alcohol use, no IV drug abuse, no anorexigen use, no HIV risk factors |
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History
| | Diagnosed with systemic sclerosis 5 years ago |
| | Referred by her rheumatologist |
| — | Matted telangiectasia |
| — | Raynaud's phenomenon |
| — | Gastroesophageal reflux disease |
| — | Dry, cracking skin on digits but without true ulcerations |
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Symptoms
| | Some dyspnea on exertion: |
| — | Climbing 2 or 3 flights of stairs |
| — | Walking up inclines |
| — | Carrying heavy packages |
| | Some fatigue after "hard day" at work |
| | Occasional palpitations with exertion |
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Reasons to investigate PAH
| | Long-standing limited SSc |
| | Progressive exertional dyspnea and fatigue |
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Physical examination
| | Vital signs: BP 141/88 mm Hg; HR 83 bpm; RR 16 bpm; SaO2 98% (room air) |
| | CV: RRR, S1S2 no murmur, rub, or gallop |
| | Lungs: clear without rales, rhonchi, or wheeze |
| | Neck: no JVD |
| | Extremities: |
| — | Multiple matted telangiectasia on face, arms, chest |
| — | No clubbing, cyanosis, or edema |
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Chest x-ray findings
| | Normal pulmonary arteries |
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Laboratory findings
| | CBC unremarkable |
| — | Hgb within normal limits |
| — | Hct within normal limits |
| — | WBC within normal limits |
| | Chem-7 |
| — | Cr: 0.6 mg/dL (normal 0.8 to 1.4 mg/dL1) |
| — | BUN within normal limits |
| — | Na+ within normal limits |
| — | K+ within normal limits |
| — | Cl– within normal limits |
| | LFTs |
| — | ALT: 14 IU/L (normal range varies)2 |
| — | AST: 25 IU/L (normal 10 to 34 IU/L)3 |
| | ESR: 17 mm/hr (normal <30 mm/hr)4 |
| | HCO3: 24 mEq/L (normal 20 to 29 mEq/L)5 |
| | ANA positive, 1:256 |
| | Scl-70 negative |
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Other findings
| | PFTs |
| — | FEV1: 94% predicted |
| — | LC: 80% predicted |
| — | FVC: 85% predicted |
| — | DLCO: 54% predicted |
| — | FEV1/FVC: 0.85 |
| — | % FVC/% DLCO: 1.6 |
| | 6-minute walk test |
| — | 470 m |
| — | Borg dyspnea index: 1 |
| | V/Q scan: low probability of thromboembolic disease |
| | HRCT chest: normal without ILD or PD |
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Echocardiogram findings
| | Rest echo |
| — | Significant dilation; PAP estimate of 40 mm Hg |
| | Exercise echo |
| — | RV strain with a mildly decreased RVEF |
| | ECG: normal sinus rhythm without RAE, RVH, or ischemic changes with exercise |
| | BP |
| — | Rest: 120/70 mm Hg |
| — | Exercise: 160/80 mm Hg |
| | PAP |
| — | Rest: 37 mm Hg |
| — | Exercise: 44 mm Hg |
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Tracleer is indicated for the treatment of pulmonary arterial hypertension (PAH, WHO Group I) in
patients with WHO Class II-IV symptoms, to improve exercise ability and decrease the rate of
clinical worsening. Patients with WHO Class II symptoms showed reduction in the rate of clinical
deterioration and a trend for improvement in walk distance. Physicians should consider whether
these potential benefits are sufficient to offset liver injury in WHO Class II patients, which may
preclude future use as their disease progresses.
Important safety information
Because of the associated risks, Tracleer may be prescribed only through the Tracleer Access Program.
Potential for serious liver injury (including, after prolonged treatment, rare cases of liver failure
and unexplained hepatic cirrhosis in a setting of close monitoring)—Liver monitoring of all patients
is essential prior to initiation of treatment and monthly thereafter.
High potential for major birth defects—Pregnancy must be excluded and prevented through the use of
reliable forms of birth control; monthly pregnancy tests should be obtained.
Contraindicated for use with cyclosporine A and glyburide.
Please see full prescribing information including BOXED WARNING.
