Pulmonary Arterial Hypertension (PAH) is a rare disease sometimes associated with collagen vascular diseases, usually Limited Scleroderma (CREST Syndrome). Pulmonary Veno-occlusive Disease (PVOD) is an uncommon subtype of PAH with a particularly poor prognosis. PVOD is usually idiopathic, however it has also been associated with chemotherapy exposure and rheumatic diseases. PVOD has rarely been associated with CREST Syndrome, however few clinical data exist concerning this connection. We present clinical, radiographic, hemodynamic and pathologic data on two patients with PVOD and associated CREST Syndrome.
Two patients managed at our institution for PVOD with concomitant CREST Syndrome were studied via retrospective chart analysis. All available clinical, radiographic, hemodynamic, and pathologic data were reviewed.
Two female patients (aged 63 and 71 years) were diagnosed with CREST Syndrome (8 and 20 years) prior to onset of pulmonary hypertension symptoms or diagnosis. Both were treated with nifedipine, prednisone, and cyclophosphamide for scleroderma, and warfarin, oxygen and epoprostenol for PAH. One received sitaxsentan prior to epoprostenol initiation. Both presented with dyspnea, (WHO Class III), accentuated P2 heart sound, RV heave, and II/VI tricuspid regurgitation murmurs. Both were seropositive for anticentromere antinuclear antibodies (titres: 1:1280 and 1:2048). Thoracic CTs demonstrated very mild interstitial Lung Disease (ILD). PFTs showed mild reductions in TLC with marked reductions in DLCO. Right heart catheterization showed: mean PAP 53 and 63 mmHg, PCWP 14 and 7 mmHg, CO 3.1 and 2.2 L/min, MVO2 44 and 37%, RAP 14 and 9 mmHg, respectively. Clinical courses were notable for rapid clinical deterioration (death within several weeks) characterized by progressive right heart failure, pleural effusions and pulmonary edema when given epoprostenol. Autopsies demonstrated PVOD without significant ILD in both cases.
PVOD and CREST Syndrome are two relatively rare conditions which may co-exist. The association carries a particularly poor prognosis in these cases, marked by clinical deterioration upon initiation of epoprostenol.
Clincians should consider PVOD in patients with scleroderma and pulmonary hypertension, particularly if thoracic CT scanning suggests ILD.
William Mansfield, Grant monies (from industry related sources) Encysive, LP Myogen Biopharmaceutical; Product/procedure/technique that is considered research and is NOT yet approved for any purpose. sitaxsentan.