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Hospital Mútua de Terrassa Barcelona, Spain
Correspondence to: Pedro Almagro, MD, Department of Medicine, Hospital Mútua de Terrassa, PI, Doctor Robert, 5, Terrassa, Barcelona, Spain 08221; e-mail: 19908pam{at}comb.es
To the Editor:
We read with interest the article by Holcomb and colleagues (December 2000)1 concerning pulmonary veno-occlusive disease (PVOD). We wish to make the following observations.
We agree with the authors that the association of symptoms of digital clubbing and primary pulmonary hypertension (PPH) should suggest the diagnosis of PVOD. This association, however, is not new. As early as 1976, Wagenvoort2 specifically pointed out that digital clubbing was part of the PVOD clinical picture.
We also agree with the authors that the diagnosis of PVOD can be suspected by using clinical and radiologic data, because these data are different from those found in patients with PPH due to pulmonary arteriopathy. We disagree, however, with the authors’ conclusion that only the presence of these clinical and radiologic data can assure the diagnosis of PVOD and that, therefore, pulmonary biopsy can be avoided. Only histologic analysis allows us to distinguish between PVOD from another unusual cause of PPH, pulmonary capillary hemangiomatosis (PCH). In patients with PCH, the symptoms of digital clubbing, hemodynamic findings, and pulmonary infiltrates are similar to those symptoms described in patients with PVOD.3 Making the correct diagnosis is important, because treatment with interferon has been proposed as a specific therapy, and the use of prostacyclins is contraindicated in PCH.4 5
Finally, in the study by Holcomb and colleagues,1 a patient with hemodynamic findings suggestive of PVOD, but without other clinical and radiologic findings, was excluded from the analysis. Obviously, if patients without suggestive clinical and radiologic findings are excluded, the patients under study will always have these abnormalities present. The possibility of PVOD, even in the absence of these findings, always exists. It would of great interest to know about the histopathologic findings of this subgroup. Only then would the conclusions of the study by Holcomb and colleagues be well-founded.
References
Vanderbilt University Nashville, TN
Correspondence to: Barry W. Holcomb, MD, Center for Lung Research, Vanderbilt University, Room T-1217 MCN, Nashville, TN 37232; e-mail: barry.holcomb{at}mcmail.vanderbilt.edu
To the Editor:
We appreciate the comments of Drs. Almagro, Alvarez, and Garau and the opportunity to respond. We searched MEDLINE using the headings "clubbing" and "pulmonary veno-occlusive disease" but found no matches. Almagro and colleagues are correct; Wagenvoort1 did comment on clubbing in pulmonary veno-occlusive disease (PVOD), but he cited no references in his review. Recently, we learned of an additional case report of clubbing in a patient with PVOD.2 More importantly, our series demonstrated clubbing in 5 of 11 patients, suggesting that clubbing is not rare in PVOD.3
The corespondents also point out that clubbing and similar radiographic
changes occur in patients with pulmonary capillary hemangiomatosis
(PCH), and they argue that it is important to differentiate PVOD from
PCH, the latter of which they believe can be treated. However, no
effective medical therapy has ever been proven for either disorder.
They cite a case report describing successful treatment of pulmonary
hemangiomatosis with interferon-
therapy, in a 12-year-old
patient.4
This patient had minimal pulmonary hypertension
with a mean pulmonary artery pressure of 20 mm Hg, far less than that
of our series.
Our experience with interferon- in the treatment of patients with
biopsy-proven PCH differs from that above: we have seen no improvement,
but we have noted significant side effects. Because no additional
studies have been published in the intervening decade to support the
efficacy of interferon as a treatment for PCH, we suspect that other
clinicians may have had similar experiences. In an informal survey of
investigators at some of the major centers in the United States, few
people have seen a case of PCH, let alone treated it. Even the authors
who described the ineffectiveness of epoprostenol therapy as a
treatment for patients with PCH6
have treated only one PCH
patient with interferon without any success (personal communication,
April 2001; M. Humbert, MD). Have Almagro and colleagues effectively
treated a case of PCH with interferon?
We agree that histologic analysis of lung tissue remains the "gold standard" for diagnosis of PVOD. However, the risks of biopsy are substantial in patients with significant pulmonary hypertension.7 Both PVOD and PCH are extremely rare illnesses, and lung biopsy may subject patients to unnecessary morbidity and mortality while delaying effective therapy with lung transplantation. Therefore, given the fact that there is no known effective medical treatment for either disorder, the risks of biopsy may significantly outweigh any benefit. Distinguishing PVOD from interstitial lung disease (which does require biopsy) may be a more relevant discussion, as some patients with interstitial lung disease do respond to medical treatment.
Regarding the exclusion of a patient with hemodynamic findings consistent with PVOD, but no other findings suggestive of the diagnosis, the point of the correspondence is not clear. The intention of our series was to raise awareness of PVOD among clinicians, since the use of vasodilators can be life threatening, and to highlight that there are frequently clues to suggest the diagnosis. Our series was not intended to estimate the incidence of PVOD in patients with pulmonary hypertension. We included only those patients with a firm diagnosis of PVOD (9 of 11 with histologic tissue biopsies for confirmation). The patient in question had no other findings to suggest PVOD, including a CT scan of the chest, which has previously been reported to demonstrate findings in PVOD quite different from those in precapillary pulmonary hypertension.8 We agree that the possibility of PVOD exists even without the characteristic findings described in our series. However, we suspect that this is uncommon, as we have not diagnosed PVOD, by histologic evaluation, in any patient in the absence of increased septal markings. Therefore, we do not understand why Almagro and colleagues feel that excluding cases with uncertain diagnosis detracts from our findings.
References
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