Chest
Volume 124, Issue 5, November 2003, Pages 2017-2022
Journal home page for Chest

Selected Reports
Pulmonary Capillary Hemangiomatosis With Atypical Endotheliomatosis*: Successful Antiangiogenic Therapy With Doxycycline

https://doi.org/10.1378/chest.124.5.2017Get rights and content

We report here our experience in achieving remission in a 20-year-old man with pulmonary capillary hemangiomatosis (PCH) with atypical endotheliomatosis following therapy with doxycycline. PCH is a rare disorder characterized by proliferating capillaries that invade the pulmonary interstitium and alveolar septae, and occlude the pulmonary vasculature. The patient’s symptoms, lung function, and radiographic findings had worsened despite treatment with both prednisone and α-interferon. He was considered to be a candidate for transplantation. Given the elevated levels of basic fibroblast growth factor (bFGF) in urine and the capillary proliferation noted on biopsy specimens, we elected to treat the patient with doxycycline, a matrix metalloproteinase and angiogenesis inhibitor. Following several weeks of therapy, a gradual resolution of symptoms was noted, with normalization of pulmonary function test results and urine bFGF levels. After 18 months of therapy, the patient remains in complete remission.

Section snippets

Case Report

Over 6 months, a previously healthy 20-year-old male college student developed hemoptysis, cough, hoarseness, and sore throat. Symptoms responded partially to therapy with oral ampicillin that was prescribed for possible sinusitis. Initially, the patient continued normal activities and denied any fatigue or dyspnea, but eventually minimal physical activity severely exhausted him, and his hemoptysis increased.

A physical examination revealed a pale, chronically ill-appearing individual without

Results

Within weeks of initiating doxycycline therapy, the patient reported a substantial increase in exercise tolerance and a decrease in hemoptysis. The prednisone dose was decreased, and the patient was weaned approximately 4 months after starting therapy. After 6 months of doxycycline therapy, the α-interferon dose was decreased. The patient stopped taking the α-interferon on his own 2 months later. Nine months after starting doxycycline therapy, the patient was symptom-free. Improvement was seen

Discussion

To our knowledge, this case of PCH with atypical endotheliomatosis is the first case of PCH, or any other vascular malformation or neoplasm, that may have been treated successfully with doxycycline. This is one of only two well-documented cases in which a patient has responded to medical therapy. In the earlier case, α-interferon therapy was discontinued after 5 years without disease recurrence (CW White; personal communication; October 10, 2000). While α-interferon therapy has been shown in

ACKNOWLEDGMENT

The authors thank Judah Folkman for his thoughtful comments on this case, and his laboratory for performing urinary bFGF enzyme-linked immunosorbent assays. We thank the laboratory of Marsha Moses for performing the urinary zymography.

References (31)

  • Massachusetts General Hospital

    Case records of the Massachusetts General Hospital: weekly clinicopathological exercises; case 38–2000—A 45-year-old woman with exertional dyspnea, hemoptysis, and pulmonary nodules

    N Engl J Med

    (2000)
  • GC Blobe et al.

    Role of transforming growth factor beta in human disease

    N Engl J Med

    (2000)
  • KN Cowan et al.

    Complete reversal of fatal pulmonary hypertension in rats by a serine elastase inhibitor

    Nat Med

    (2000)
  • KN Cowan et al.

    Elastase and matrix metalloproteinase inhibitors induce regression, and tenascin-C antisense prevents progression, of vascular disease

    J Clin Invest

    (2000)
  • C Domingo et al.

    Pulmonary capillary hemangiomatosis: report of a case and review of the literature

    Respiration

    (1992)
  • Cited by (0)

    Dr. Roberts was supported by a Nirenberg Center for Advanced Lung Diseases Fellowship, and Dr. Marler was the Garrett Smith Fellow in the laboratory of Dr. Judah Folkman.

    View full text