Pulmonary capillary hemangiomatosis is a rare cause of pulmonary hypertension. First described by Wagenvoort et al. in 1978, its’ etiology and pathogenesis remains uncertain (1). In most cases, this disorder is elusive until necroscopy. We describe a unique case with a family history of pulmonary hypertension, in which two distinct sets of pathology were attained and several therapeutic agents were administered.
A thirty-four year old previously healthy Caucasian man presents with severe dyspnea and chest pain a few days after cleaning out his garage. A strong family history of pulmonary hypertension (paternal grandmother, father, two paternal aunts) was noted. Clinical exam was significant only for an audible P2. High resolution computed tomography demonstrated bilateral diffuse reticulonodular infiltrates. Laboratory data was unremarkable aside from significant hypoxemia with an elevated alveolar-arterial gradient. Cardiac catherization showed preserved left ventricular function with patent arteries, a pulmonary artery pressure of 59/22 with a wedge pressure of 8. Pulmonary function tests demonstrated reduced diffusion capacity(30% predicted). Bronchoscopy with lavage revealed 69% histiocytes, 27% lymphocytes, 4% neutrophils and hemosiderin laden macrophages. An open lung biopsy displayed morphologic evidence of pulmonary hypertension and prominent arterial thromboembolic changes with some venous involvement. Therapy included oxygen, steroids and anticoagulation with initial clinical improvement. Persistent pulmonary hypertension prompted a vasoreactivity study and initiation of epoprostenol. Patient’s clinical course included several hospitalizations for infection, with a positive response to antibiotics and high dose steroids. Bilateral lung transplantation was performed 11 months after initial presentation. Explant lung pathology was consistent with pulmonary capillary hemangiomatosis.
Pulmonary capillary hemangiomatosis is a rare cause of pulmonary hypertension. The reported age ranges from 5 to 71, with a peak between 20 and 40 years. No gender prevalence is noted. Most cases are sporadic; however, a hereditary form with possible autosomal-recessive inheritance was reported(2). Histologically, the disorder is characterized by proliferation of small capillaries within the pulmonary interstitum and invasion of vessel and airway walls. Patients usually present with progressive dyspnea, cough, pulmonary hypertension, hemoptysis and bilateral reticulonodular infiltrates. Typically, death ensues 1 to 5 years after onset of symptoms. Although presentation, imaging, and other studies may be suggestive, a strong clinical suspicion and tissue is needed for diagnosis. Successful treatment includes lung transplantation or pneumonectomy. Interferon-alpha and doxycycline have provided positive results in case reports; however, glucocorticoids have no proven role. Vasodilators such as calcium channel blockers and epoprostenol have resulted in lethal results and are “contraindicated”(3).In comparison, our case presents several unique aspects. Two individual open lung biopsies (obtained eleven months apart) demonstrated distinct histologies. The first was suggestive of chronic small vessel thromboembolic pulmonary hypertension, and the second clearly showed pulmonary capillary hemangiomatosis. One may propose that the initial pathology may be an early presentation of the disease. In terms of therapy, our patient initially responded positively to steroids and his course was ameliorated with epoprostenol. It is possible that his initial pathology and response to therapy stems from an earlier presentation as compared to other reported cases. Finally, the history of pulmonary hypertension in three generations suggests a hereditary form of the disease.
Pulmonary capillary hemangiomatosis is a rare disease. Our case suggests that “early” pulmonary capillary hemangiomatosis may only display features of small vessel thromboembolic pulmonary hypertension. Although an objective and subjective improvement with moderate-high dose steroids was noted initially, a rapidly progressive course ensued. Interestingly, the cardiopulmonary status did not worsen on epoprosternol. Although major aspects of the disease have been characterized, a complete definition of the disease process continues to evolve.
Saadia Faiz, None.