Relapse of respiratory insufficiency one year after organising pneumonia.

A 66-yr-old nonsmoker female presented in June 1998 with a 3-week history of progressive dyspnoea. Her past medical history was noteworthy for idiopathic bilateral uveitis without sequelae between 1978 and 1993, hypercholesterolaemia and angina pectoris. Her medication included fluvastatin, which started in June 1997 (60 mg?day-1), trinitrin patch and acetylsalicylic acid. She had no particular work-related or environmental dust exposure. Examination of the chest revealed bilateral basal crackles. There was no digital clubbing or extrapulmonary signs. At admission, chest radiograph and thoracic computed tomography (CT) scan (fig. 1a) revealed patchy consolidations, predominantly involving the bilateral lower lobes. Arterial blood gas analysis under supplemental oxygen (6 L?min-1) revealed a partial pressure of oxygen of 8.3 kPa (62 mmHg), a partial pressure of carbon dioxide of 4.3 kPa (32 mmHg) and a pH of 7.46. As a result of rapidly progressive respiratory failure, she was admitted to the intensive care unit. Bronchoalveolar lavage (BAL) analysis showed a 1406106 cells?L-1 increased lymphocyte rate (38%), low CD4/CD8 ratio (0.18), increased neutrophil rate (17%), 44% of macrophages and 1% of eosinophils. Transbronchial lung biopsy, including 20 alveoli, revealed typical lesions of organising pneumonia (fig. 2a). Minor salivary gland biopsy specimens found no sign of Sjögren9s syndrome. Antinuclear antibodies were positive at a titre of 1:50 of speckled pattern; anti-JO1 antibodies were positive. Neither clinical nor biological signs of muscular involvement were found. Fluvastatin was stopped. Oral steroid therapy was initiated (1 mg?kg?day prednisone) and was progressively reduced. The evolution was frankly favourable assessed by clinical symptoms, radiology (fig. 1b) and pulmonary function tests (PFT). In September 1999, whilst the corticosteroid dose was tapered to 7 mg?day, the patient experienced relapsing severe respiratory insufficiency and basal opacities on the chest radiograph. Despite 2 months of high-dose prednisone (1 mg?kg?day), no improvement was obtained. Physical examination revealed inspiratory crackles without digital clubbing or extrapulmonary signs. A chest radiograph showed bilateral pulmonary retraction and reticulonodular opacities that were predominant in the lower lobes. PFT revealed a restrictive ventilatory defect, decreased carbon monoxide diffusing capacity of the lung and arterial hypoxaemia necessitating continuous oxygenotherapy. AntiJO1 antibodies were positive without muscular involvement. BAL analysis showed 170610 cells?L with 84% of macrophages, 14% of lymphocytes (CD4/CD8: 0.22), 1.5% of neutrophils and 0.5% of eosinophils. Thoracic high-resolution CT scans were performed (fig. 1c) and the finding resulted in the patient undergoing an open lung biopsy (OLB).