Anti-Jo-1 Antibody-positive Interstitial Pneumonia in an Elderly Patient with Congenital Rubella Syndrome

Abstract

A 71-year-old woman with congenital rubella syndrome (CRS) presented with prolonged cough. No physical findings suggested the presence of any connective tissue diseases. Chest computed tomography showed ground-glass opacities and consolidations in the bilateral lower lobes. She had elevated serum Krebs von den Lungen-6, hypoxemia and positive serum anti-Jo-1 antibody. Bronchoalveolar lavage fluid revealed lymphocytosis with a decreased CD4/CD8 ratio. A transbronchial lung biopsy specimen revealed organizing pneumonia. Based on a diagnosis of interstitial pneumonia with autoimmune features (IPAF), systemic corticosteroids were administered, and a good outcome was obtained. A possible relationship between CRS and IPAF is herein discussed.

Keywords: autoimmunity; congenital rubella syndrome; interstitial pneumonia.

Figure 1.

Physical features of the patient. Right eye and whole-body images show the presence of a cataract (A), dwarfism and microcephaly (B, arrow).

Figure 2.

Chest radiography findings at the initial presentation. Ground-glass shadows are observed in the bilateral lower lung fields (A). Chest CT shows ground-glass opacities and consolidations in the bilateral lower lobes (B) and small volumes of bilateral pleural effusion and pericardial effusion (C).

Figure 3.

A transbronchial lung biopsy specimen of the left lower lobe. An image shows alveolar septal thickening, inflammatory cell infiltration, and organizing pneumonia (Hematoxylin and Eosin staining, ×100).