Pulmonary-Renal Syndrome with Negative ANCAs and Anti-GBM Antibody

Abstract

We report the case of a 76-year-old woman who was referred to our hospital for a gradually worsening cough and renal dysfunction. Although pneumonia was initially suspected, imaging findings of the lungs revealed diffuse alveolar hemorrhage at a later date. Renal failure developed and hemodiafiltration was performed on the 9th day. Rapidly progressive glomerulonephritis with crescent formation was diagnosed by renal biopsy. This case presentation has important clinical implications because uncategorizable pulmonary-renal syndrome (PRS) without the presence of ANCAs and anti-GBM antibody is extremely rare and has high rates of morbidity and mortality. No treatment has been established.

Figure 1

The patient's clinical course.

Figure 2

(a), (b) Chest X-ray and CT on admission showed bilateral central opacities, which are more confluent in the right upper and lower lung zone than elsewhere. There is slight cardiomegaly. (c) The radiograph obtained after intubation on the 10th day showed increased confluence of the opacities. The right hemidiaphragm is partially obscured. (d) Chest CT scan on the 10th day showed bilateral ground-glass opacities corresponding to the central and perihilar opacities and both small pleural effusions. (e) Chest CT scan on the 36th day showed progressed bilateral ground-glass opacities, confluent, and consolidated opacities, and a large quantity of pleural effusions in both lung fields. (f) Chest X-ray on the 92nd day showed the disappearance of infiltrative shadows in both lung fields.

Figure 3

(a), (b), (c) Light-microscopy examination of renal-biopsy specimens showed proliferative-appearing glomeruli with focal formation of cellular and fibrocellular crescents. The GBM was duplicated. Many glomeruli were globally sclerosed, indicating disease chronicity. (A periodic acid Schiff staining, B periodic acid silver-methenamine staining, C Masson's trichrome staining). (d) Immunofluorescence microscopy showed slightly bright granular staining for C3 in the mesangium area. (e) Immunofluorescence microscopy showed weak staining with IgG in a linear pattern in part of the GBM. GBM: glomerular basement membrane.