A case of pulmonary veno-occlusive disease respondong to treatment with azathioprine.

Sanderson, John E.; Spiro, S. G.; Hendry, A.; Turner-Warwick, M

doi:10.1136/thx.32.2.140

Cited by 61 publications

(23 citation statements)

References 13 publications

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“…In addi tion, the recent case report of a patient with PVO who had immune complex de position on the basement membranes of small pulmonary venules [11] raises the possibility of an immunologic etiology for the disease. Additional support for such a mechanism comes from a case report of a patient with arthralgias, Raynaud's phen omenon, a positive test for antinuclear an tibody, but no evidence of immune com plex deposition, who showed marked im provement of her PVO after treatment with azathioprine and prednisone [9], Patients suffering from PVO generally present with fatigue and dyspnea on exer tion which progresses to signs and symp toms of pulmonary hypertension. Since the presentation is rather nonspecific the differential diagnosis is long.…”

Section: Discussionmentioning

confidence: 99%

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Pulmonary Veno-Occlusive Disease in the Middle-Aged

Glassroth¹,

Woodford²,

Carrington³

et al. 1985

Respiration

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Pulmonary veno-occlusive disease (PVO) is an uncommon cause of pulmonary hypertension which is difficult to diagnose without histology. We report 3 cases of histologically confirmed PVO. Our patients were all middle-aged or older, and 1 had spontaneous improvement in his symptoms suggesting that PVO may occur more commonly among older adults than previously believed and that indolent forms of the disease may exist. As new therapeutic approaches to the various entities causing pulmonary hypertension are developed, it is increasingly important to determine precisely which condition is present in a given patient.

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Section: Discussionmentioning

confidence: 99%

“…3, and possibly case No. 1, have only rarely been reported [9], There is currently no known effective therapy though the role of immunosuppressive therapy bears further study [9], The etiology of PVO remains unknown. One popular hypothesis is that an antece dent viral infection initiates the vascular damage [2].…”

Section: Discussionmentioning

confidence: 99%

Pulmonary Veno-Occlusive Disease in the Middle-Aged

Glassroth¹,

Woodford²,

Carrington³

et al. 1985

Respiration

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“…The basis for the use of immunosuppressive agents in some selected PAH patients was initially anecdotal, with rare reports of clinical and haemodynamic improvements in PAH and PVOD patients, suggesting the use of corticosteroids, cyclophosphamide and azathioprine [19,27,[68][69][70][71]. PVOD is also thought to be present in some cases of pulmonary hypertension patients displaying sarcoidosis.…”

Section: Immunosuppressive Therapymentioning

confidence: 99%

Pulmonary veno-occlusive disease

Montani¹,

Price²,

Dorfmüller³

et al. 2008

Eur Respir J

272

250

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Pulmonary veno-occlusive disease (PVOD) is currently classified as a subgroup of pulmonary arterial hypertension (PAH) and accounts for 5-10% of cases initially considered to be idiopathic PAH. PVOD has been described as idiopathic or complicating other conditions, including connective tissue diseases, HIV infection, bone marrow transplantation, sarcoidosis and pulmonary Langerhans cell granulomatosis. PVOD shares broadly similar clinical presentation, genetic background and haemodynamic characteristics with PAH. Compared to PAH, PVOD is characterised by a higher male/female ratio, higher tobacco exposure, lower arterial oxygen tension at rest, lower diffusing capacity of the lung for carbon monoxide, and lower oxygen saturation nadir during the 6-min walk test. High-resolution computed tomography (HRCT) of the chest can be suggestive of PVOD in the presence of centrilobular ground-glass opacities, septal lines and lymph node enlargement. Similarly, occult alveolar haemorrhage is associated with PVOD. A noninvasive diagnostic approach using HRCT of the chest, arterial blood gases, pulmonary function tests and bronchoalveolar lavage could be helpful for the detection of PVOD patients and in avoiding high-risk surgical lung biopsy for histological confirmation. PVOD is characterised by a poor prognosis and the possibility of developing severe pulmonary oedema with specific PAH therapy. Lung transplantation is the treatment of choice. Cautious use of specific PAH therapy can, however, be helpful in some patients.KEYWORDS: Alveolar haemorrhage, BMPR2, computed tomography, diffusing capacity of the lung for carbon monoxide, pulmonary arterial hypertension, pulmonary veno-occlusive disease P ulmonary arterial hypertension (PAH) is a severe condition characterised by elevated pulmonary artery pressure leading to right heart failure and death [1,2]. Pulmonary veno-occlusive disease (PVOD) is classified as a subgroup of PAH and accounts for 5-10% of histological forms of cases initially considered to be idiopathic PAH. Even though the first welldocumented case of PVOD was described .70 yrs ago, the characteristics and pathophysiology of this disease remain poorly understood [3,4]. While pulmonary vascular pathology of idiopathic or familial PAH is characterised by a major remodelling of small pre-capillary pulmonary arteries with typical plexiform and/or thrombotic lesions, PVOD preferentially affects the post-capillary venous pulmonary vessels [5,6]. Despite this anatomical histological difference, PVOD has a very similar clinical presentation to PAH but is characterised by a worse prognosis and the possibility that severe pulmonary oedema can develop with specific PAH therapy, justifying the importance of diagnosing this disease. A definitive diagnosis of PVOD requires histological analysis of a lung sample [7,8]; however, surgical lung biopsy is a high-risk procedure in these patients and the development of a less invasive diagnostic approach would be preferable [9][10][11]. The present manuscript will summa...

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“…This woman's DLco was 17% of predicted even though spirometry, lung volume measurements, and the chest roent genograms were normal. Previous studies of patients with pulmonary veno-occlu sive disease have documented a range of DLco from 29 to 59% of predicted [3,4,11,12], In some instances, pulmonary venous occlusion has been associated with granu lomatous inflammation and an interstitial infiltrate [12,13]. In these circumstances, a reduced Dlco may reflect changes in the gas exchange surface which result from granulomatous and interstitial inflamma tion.…”

Section: Discussionmentioning

confidence: 99%

Pulmonary Veno-Occlusive Disease Associated with Severe Reduction of Single-Breath Carbon Monoxide Diffusing Capacity

Tv²,

Hill

1988

Respiration

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A 49-year-old woman presented with pulmonary hypertension, profound arterial hypoxemia, and a single-breath carbon monoxide diffusing capacity (D_Lco) which was 17% of predicted. History, physical examination, and chest roentgenograms did not suggest the presence of parenchymal pulmonary disease. Spirometry and lung volume measurements were within normal limits. Pulmonary veno-occlusive disease was diagnosed by lung biopsy. This case illustrates the severe reduction of D_Lco which can be associated with pulmonary veno-occlusive disease.

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A case of pulmonary veno-occlusive disease respondong to treatment with azathioprine.

Cited by 61 publications

References 13 publications

Pulmonary Veno-Occlusive Disease in the Middle-Aged

Pulmonary Veno-Occlusive Disease in the Middle-Aged

Pulmonary veno-occlusive disease

Pulmonary Veno-Occlusive Disease Associated with Severe Reduction of Single-Breath Carbon Monoxide Diffusing Capacity

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