05 February 2018 by Gul Ongen

Can idiopathic lymphoid interstitial pneumonia be a cause of moderate pulmonary hypertension?


Lymphocytic interstitial pneumonia (LIP) is a syndrome of fever, cough, and dyspnea, with bibasilar pulmonary infiltrates consisting of dense interstitial accumulations of lymphocytes and plasma cells. Most cases of LIP are associated with other conditions and idiopathic cases are rare. Although LIP is one of the interstitial lung diseases, it is not known as a frequent cause of pulmonary hypertension (PH).


A 62-year-old womanpresented with shortness of breath, dry cough for 5 years and treated for asthma since then. Physical examination revealed bilateral basal crackles. Chest CT showed dilated pulmonary artery diameter and right heart chambers, hilar lymph nodes (12 and 15 mm), mozaic pattern, ground-glass opacities and traction bronchiectasis, Thickening of the bronchovascular bundles and interlobular septal thickening and reticular densities with bilateral lower level predominance. Auto-antibodies, IgG immunoglobulins, Raynaud, HIV and EBV were negative. Spirometric lung function tests were normal, but low diffusion capacity (DLCO 35% ; DLCO/VA 48%) and resting hypoxaemia. Bronchoalveolar lavage showed increased lymphocytes (50%) , neutrophils (10%), CD4 (89%), CD8 (10.8%) and CD4/CD8: 8.25. RHC revealed PABm 44mmHg (65/30); wedge pressure 14mmHg; PVR 4.8 W;CI 3.28L/m/m2. The patient recently started to receive corticosteroid therapy.Follow-up data of both diseases will be shared soon.


In a PH patients with CT findings described above, LIP should be considered.

Key Contributors

Kılıçlıran Avcı B. Atahan E, Erben C, Akman C, Öngen Z, Öngen HG. İstanbul University, Cerrahpaşa Medical Faculty, İstanbul, Turkey.

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