Pulmonary Veno-Occlusive Disease in Rheumatoid Arthritis: A Rare Pathological Entity Independent of Interstitial Lung Disease

TL;DR

A rare lung disease called PVOD was found in a rheumatoid arthritis patient without interstitial lung disease, suggesting PVOD can occur independently.

Contribution

PVOD is identified as a distinct pathological entity in rheumatoid arthritis patients, independent of interstitial lung disease.

Findings

Postmortem examination revealed PVOD features in a rheumatoid arthritis patient.

PVOD was confirmed as the direct cause of death due to pulmonary edema.

No evidence of RA-associated interstitial lung disease was found in the patient.

Abstract

We present the case of an 83-year-old woman with a long-standing history of rheumatoid arthritis (RA) who was found collapsed at home. The patient presented with cardiopulmonary arrest and could not be resuscitated. A postmortem examination was performed to determine the cause of death. Postmortem computed tomography (CT) ruled out intracranial hemorrhage but revealed diffuse bilateral pulmonary consolidations and signs of bronchial obstruction. The autopsy revealed severe pulmonary edema and marked right ventricular hypertrophy. Microscopic examination of the lungs demonstrated characteristic features of pulmonary veno-occlusive disease (PVOD), including widespread fibrous intimal thickening and occlusion of small pulmonary veins and venules. Notably, there was no evidence of RA-associated interstitial lung disease (ILD). The direct cause of death was identified as pulmonary edema secondary to PVOD. This case highlights that PVOD can occur in patients with RA as a distinct pathological entity, independent of ILD. This finding is significant as it contrasts with previous reports where PVOD was associated with ILD. Therefore, clinicians should consider PVOD in the differential diagnosis of RA patients who present with unexplained pulmonary hypertension or progressive dyspnea, even in the absence of interstitial lung disease.

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