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Author: Allison K. Nitsch, Texas A&M Health
Science Center - College of Medicine, Class of 2011
Introduction: Pulmonary hypertension (PH) is a
progressive disease with multiple etiologies. Management of PH is
tailored to the origin of the disease, with therapy for some forms
actually worsening the clinical manifestations of other subtypes.
Pulmonary capillary hemangiomatosis (PCH) is a rare, but rapidly
fatal cause of PH. Our case highlights how PH due to PCH is often
misdiagnosed until autopsy.
Case Presentation: A 40-year-old African
American female presented to the emergency department with
complaints of worsening chronic dyspnea over the previous week. Her
past medical history was significant for dermatomyositis and PH
diagnosed with echocardiogram, which demonstrated right ventricular
systolic pressures of 40 mmHg. Her PH had previously been
attributed to her connective tissue disease. Her condition had
continued to progress despite therapy with bosentan and sildenafil.
Previous right heart catheterization revealed pulmonary artery
systolic pressure of 81 mmHg, diastolic pressure 33 mmHg, and
capillary mean pressure of 10 mmHg. On admission, CT scan of the
chest demonstrated bilateral ground glass opacities with diffuse
interlobular septal thickening and mediastinal adenopathy. The
patient's condition rapidly deteriorated with increasing dyspnea
and hypoxia. The patient required transfer to the ICU where she
succumbed to her illness within 24 hours of admission. Autopsy
ruled the primary cause of death to be progressive PH secondary to
PCH. Microscopic analysis of lung tissue revealed pulmonary
Discussion: Pulmonary capillary hemangiomatosis
(PCH) is a rare cause of PH with fewer than 50 cases reported in
the literature. PCH is marked by capillary proliferation within the
alveolar walls and respiratory epithelium leading to PH. Other
pathological findings include interstitial lymphocytic infiltrate,
capillary proliferation and interlobular septal thickening.
Etiology of PCH is unknown. PCH is similar to pulmonary
vaso-occlusive disease (PVOD) clinically and pathologically.
Consequently, some have suggested that PVOD and PCH are two
diseases in the same spectrum or that PCH develops secondary to the
congestion caused by PVOD. Clinically, PVOD and PCH are
indistinguishable from primary pulmonary arterial hypertension.
However, delineating PCH from other causes of PH is important as
treatment of PCH with IV vasodilators in some case reports have
worsened patients' clinical condition and led to pulmonary edema.
Definitive diagnosis of PCH is made through lung biopsy. Successful
outcomes have been reported with doxycycline, imatinib mesylate and
interferon-a2 treatments in the literature. However, no treatment
has been shown to improve morbidity and mortality except for lung
transplantation. The imaging and right heart catheterization
findings associated with PCH can facilitate rapid lung biopsy, lung
transplantation and perhaps cure of these patients.
July Issue of IMpact