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PCH (pulmonary capillary hemangiomatosis)
ü Incidence
– Less than 40 cases have been reported in the literature
– frequency of almost 4 cases per million individuals young adults aged 12 to 71 years (mean age, 29 years)
– predominantly adolescents, children, premature infants, and even in newborns
– affected both sexes.
ü Signs of postcapillary pulmonary hypertension
– dyspnea, cough, pleural effusion and hemoptysis with fatigue and weight loss.
– with an indolent onset of signs and symptoms slowly progressing to cor pulmonale with normal pulmonary wedge pressures
– Fever, respiratory tract infection, digital clubbing, thrombocytopenia (especially in the pediatric age group), and hemorrhagic complications, including secondary hemothorax, may occur.
ü PFT
– Obstructive , restrictive, mixed type
– Decreasing the working lung surface and the DLCO ¡é
ü median survival period :
– 3 years from the time of diagnosis because the slow progression to cor pulmonale
– right ventricular failure can eventually end in cardiovascular collapse
l pathogenesis
ü The nature and pathogenesis of PCH are still unknown.
ü Congenital abnormality.
– Vevaina and Mark reported a case of PCH affecting a 25-year-old woman with congenital scoliosis.
ü Vascular neoplasm ( reactive angioproliferation)
ü Complication of autoimmune disease and hereditary disease
– Takayasu arteritis, SLE, Kartagener syndrome, hereditary telangiectasia.
ü Severe passive congestion could be one of the causes of PCH
– unique case of PCH arising in the lung with longstanding passive congestion due to hypertrophic cardiomyopathy
ü Sequelae of pulmonary venoocclusive disease (PVOD)
– new vessels are formed to try to get around the venous obstruction.
l Image finding
ü CXR
– Diffuse reticulonodular pattern and/or increased septal lines
ü Chest CT
– bilateral diffuse, centrilobular, poorly defined nodular opacities.
– Interstitial infiltrations, thickened fissures, interlobular septa, or pleura can be seen separately or in combination.
– focal angiomatoid proliferation within bronchovascular structures along the alveolar septa and within the vessel walls.
– Lobular GGO in the area of increased pulmonary perfusion.
– As the disease progresses, the classic CT features of secondary pulmonary arterial hypertension will be manifest, including the enlargement of the main pulmonary artery and the right chamber of the heart.
l Differential diagnosis
ü PAH
– the changes caused by pulmonary arterial hypertension are limited to the arterial vasculature
– HRCT images with pathological features limited to the arterial vasculature, where the caliber of peripheral vessels is abruptly diminished.
– Centrilobular nodules, septal thickening, lymph node enlargement, or interstitial pathological features are not observed
ü PVOD
– has radiological and pathological features that are very similar to those of PCH;
– Is characterized by small areas of venous infarcts or areas of discrete congestion.
– Veins draining into interlobular septa show partial or complete occlusion, with irregular nodules of intimal fibrous thickening in the venous walls.
– A possible tool of differentiation
: the usually smooth septal thickening in the case of PVOD, whereas it may be nodular in the case of PCH.
l Diagnosis
ü Lung biopsy- open lung biopsy is helpful
– TBLB is contraindicated to avoid massive bleeding,
– The unusual distribution of the vascular network in PCH often leads to the misdiagnosis of TBLB as ILD,PVOD
ü Pathology
– the proliferation of thin-walled capillaries with a benign appearance.
– Proliferating capillaries infiltrate the lung parenchyma, blood vessels, interlobular septa, bronchial walls, pleura, and pericardium.
– dark-red patches or nodules with patchy hemorrhage in bilateral lungs.
– PCH-like foci
• no evidence to judge whether the present case represents the advanced stages of PCH or a peculiar subtype of PCH
l Treatment
ü Supportive care and symptomatic treatment with ACE inhibitors,diuretics, oxygen, and warfarin.
ü Corticosteroid
ü Pneumonectomy
– 1 pt with massive hemoptysis-> no recurrence
ü Interferon 2a
– 1 child pts -> clinical improvement and histologic regression after 30 months.
– may induce a favorable response through the inhibition of endothelial cell proliferation
ü Doxycycline
has also been shown to be of benefit
ü Epoprostenol and other Prostaglandins
– should be avoided in PCH
– worsening of hypoxemia , pulmonary edema, 3 cases of death
ü Lung transplantation
– is still considered the only definitive treatment.
– Double-lung transplants are usually indicated for patients with PPH
– Heart-lung transplants are often reserved for those with complex congenital heart disease.
l Conclusion
l PCH is clinically suggested when the patient presents pulmonary hypertension in combination with hemoptysis, a reticulo-nodular pattern on chest radiograph and focally enhanced perfusion in the lower lobes of the lung.
* reference journal ÷ºÎ ÇÕ´Ï´Ù.
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