Background Lymphocytic interstitial pneumonia is characterized by an infiltrate of mononuclear cells, mostly mature lymphocytes, restricted to the interstitium of the lungs. Lymphocytic interstitial pneumonia may progress to an end stage lung disease (characterized by non-specific interstitial fibrosis), a usually indolent condition that gradually progresses to respiratory failure.
Objectives The aim of this study was to determine: the prevalence of lethal lymphocytic interstitial pneumonia (LyPn) in rheumatoid arthritis (RA), and its histological and clinical characteristics.
Methods A randomized (non-selected) autopsy population of 234 in-patients (females 170, average age 65.4 years, range 88-16, average disease duration 15.4 years, onset of RA 49.9; males 64, average age 66.9 years, range 87-19, average disease duration 12.8 years at death, onset of RA 49.6) with RA was studied. RA was confirmed clinically according to the criteria of the ACR. Causes of death due to associated diseases, or complications of RA were histologically diagnosed post mortem. The retrospective analysis of clinical complaints was based on clinical records.
Results Lethal LyPn was observed in 3 (1.28%) of 234 RA patients.
In the lungs of three patients microscopically marked disseminated, peribronchiolar accentuated lymphoid infiltration with secondary follicles was noted. The bronchioles and terminal bronchioles appeared compressed, narrowed, and occluded. These were accompanied by lobular-sublobular atelectasis, with or without inflammation, respecting the anatomical borders. The lungs in 2 of 3 patients showed scattered irregular foci of lobular and/or sublobular pneumonia, which was not centered on terminal and respiratory bronchioles and respected more or less the anatomic borders of lobules.
In the third patient a more diffuse interstitial lymphoid infiltration was present, without peribronchial secondary follicles and prominent lobular-sublobular atelectasis and inflammation.
Conclusions The focal pneumonia due to peribronchial nodular lymphoid hyperplasia (LyPn) had no haemorrhagic character and was not accompanied by thrombembolism or vasculitis. At variance of simple bacterial bronchopneumonia, the inflammatory foci were smaller and the inflammation respected the anatomic borders of lobules (1). The lobular-sublobular infarction pneumonia caused by thromboembolism – in contrast to LyPn - has a haemorrhagic character.
In our patients focal pneumonia due to peribronchial nodular lymphoid hyperplasia was clinically accompanied by occasional febrile episodes and rapidly progressive respiratory failure. The clinical complaints were accompanied by transient (migratory) multifocal – micronodular infiltrates by X ray and reacted poorly to antibiotics. The patients died of rapidly progressive cardio-respiratory insufficiency. None of the lethal LyPns were diagnosed clinically.
Conclusion In case of multifocal pneumonia refractory to antibiotics LIPn should be keep in consideration.
Bély M, Apáthy Άgnes (1992) Changes of the lung in rheumatoid arthritis - Rheumatoid pneumonia. A clinicopathological study. Acta Morph. Acad. Sci. Hung. 39, 117-156.
Disclosure of Interest None Declared