We report a 27 year old man, 10 pack-years smoker, with a one-month history of dry cough, shortness of breath and general status's deterioration. A lung X ray showed bilateral alveolar opacities (Figure 1). Blood gases analysis in ambient air showed a pO2 of 57 mmHg, a pCO2 of 38.6 mmHg, a HCO3- of 29.2 mmHg. Because of the considerable deterioration in his respiratory condition and the need for high oxygen flow, he was referred to intensive care unit (ICU) with the diagnosis of an acute respiratory failure (ARF). Chest computed tomography (CT) showed diffuse ground-glass opacities (Figure 2A, Figure 2B) and mediastinal lymphadenopathies. All microbiological infectious investigations, especially tuberculosis, were negative. He had hypergammaglobulinemia of 21.9 g/l. Other immunological tests were otherwise negative. Transthoracic echocardiography was performed finding a hyperkinetic left ventricle, a small dilation of right ventricle with PAPs 48 mmHg and an intrapulmonary shunt revealed by contrast test. The bronchial endoscopy showed an inflammatory bronchial tree and the bronchoalveolar lavage (BAL) concluded to lymphocytic alveolitis with a ratio CD4 (helper T cells) over CD8 (suppressor T cells) (CD4/CD8) of 0.5. The bronchial biopsies showed inflammatory bronchial mucosa without any specificities. Despite the absence of Koch's bacillus in sputum analysis, we decided to begin a trial anti-tuberculosis treatment because of the severity of lung involvement added to high endemicity of tuberculosis in our country. Two weeks later, the patient presented an allergic skin reaction. As no recovery's signs were noted, we decided to stop the treatment. Corticosteroids were initiated considering idiopathic diffuse infiltrating pneumonitis. Unfortunately, the patient did not show any improvement and the chest scan performed after 27 days of treatment showed worsening interstitial infiltrates and fibrosis.
Clinically, he required high flow oxygen (14 l/mn) alternating with non invasive ventilation sessions. Histological examination of the open lung's biopsy then performed showed diffuse lung parenchyma damage. It was altered by a mutilating fibrosis that was widening the alveolar septa. This fibrosis contained an abundant inflammatory infiltrate made of lymphocytes, plasmocytes and fewer neutrophils and eosinophils. Epithelioid and giant cell granulomas were also observed without either caseous or fibrinoid necrosis. This mutilating fibrosis induced an enlargement of some alveoli resulting in the formation of a honeycomb aspect. Fibroblastic foci were also noticed. Some alveoli contained hyaline membranes that were sometimes incorporated in the septa. There were no mineral particles observed with polarized light (Figure 3). Regarding these features, the diagnosis of usual interstitial pneumonia (UIP) associated to lesions of sarcoidosis or berylliosis with aspects suggestive of an acute exacerbation was retained. Since sarcoidosis was not very likely according to CD4/CD8 ratio in BAL, we investigated risk factors for chronic beryllium disease, particularly occupation's patient history. He was working as glassblower in an artistic glass factory since six months. Further to our request, occupational department's experts inspected the workplace and confirmed beryllium existence in compounds of colored glass. After confirmation of CBD, steroids were maintained and oxygen requirement slowly decreased. The patient was finally discharged at home after 55 days steroid treatment with long term oxygen therapy (2 l/minute). Pulmonary function tests at discharge showed a severe restrictive impairement. Steroid doses were slowly decreased and stopped after 10 months. A pre-transplant assessment was also made. One year after discharge, the patient felt somewhat better and pulmonary function tests showed a slight improvement. The CT scan performed found a partial regression of lung consolidations and ground glass opacities, but increase of distortion signs, cavities appearance of which one filled by aspergilloma. He received oral voriconazole 200 mg per day during 3 months. After a two-year period from the date of CBD diagnosis, the patient was again admitted in ICU for ARF. An enhanced CT scan performed showed further deterioration of pulmonary parenchyma (Figure 2C, Figure 2D) and pulmonary embolism with fatal issue.
CT scan (lung windowing): a & b) bilateral lung consolidations and ground glass opacities with bronchial distortion; c & d) CT scan at 2 years: partial regression of lung consolidations and ground glass opacities and increase of distortion signs and cavities appearance.