A 51-year-old patient was admitted to the hospital with retrosternal pain in December 2010. In his medical history, the patient mentioned hypothyroidism and has been on L-thyroxin 150 mg/day for 13 years. The patient's medical history also revealed hypertension and mild heart failure, he had been receiving angiotensin-converting-enzyme (ACE) inhibitors (ramipril 5 mg/day) and beta blockers (bisoprolol 5 mg/day) as treatment for the past 2 years. He occasionally received a nonsteroidal anti-inflammatory drug (NSAID) for musculoskeletal pain. In order to complete the diagnostic work up, the patient underwent a coronary angiography on January 19, 2011. During the examination, just after radiographic contrast media administration, (he received 60 mL iopromide intravenous [iv]), he presented symptoms of an allergic reaction, including dyspnea, tachypnea, tachycardia, and hypoxia. In addition, his lung X-ray showed a bilateral reticular thickening, an image that agreed with a possible allergic alveolitis. The patient was treated with corticosteroids (40 mg prednisolon iv). His clinical situation promptly improved, but the X-ray findings persisted (the patient had a normal X-ray image before the examination) (Figure 1A and B). He had no prior history of allergic bronchial asthma or other allergies, and this was the first time that he received a contrast media agent.
Throughout the first 15 days, the patient's X-ray findings remained abnormal and he presented with hypoxia and desaturation at room temperature (pO2 47-49 mmHg, pCO2 37-39 mmHg [FiO2 21%]). He additionally demonstrated decreased ability for exercise (6-minute walking test [6MWT] 360 m). The echocardiogram did not show significant pathologic findings (left ventricular ejection fraction 65%, interventricular septal thickness 14 mm). However, the body plethysmography was compatible with a restrictive disorder and severe diffusion disorder (TLCO 34%, KCO 61%, TLC 3.81 [55%], FEV1 1.8 L [51%], VC max 2.2l [49%]). The high resolution computed tomography (HRCT) of the lungs revealed an eosinophilic pulmonary fibrosis pattern of airspace disease and patchy peripheral ground-glass opacities (Figure 2). The cardiopulmonary exercise test confirmed a limitation of pulmonary function (Wmax 95 W [53 W, ramp 12 W/minute]; VO2 max 1614 mL/minute [63% predicted] = 17.7 mL/minute/kg; Wmax 95 W = 43% predicted; O2 pulse demonstrated a plateau at 11.4 mL/beat [67%], VE/VO2 or VE/VCO2 under maximal workload [without any further reserve]).
Finally, we performed a right heart catheterization and we confirmed the history of a true diastolic dysfunction of the left ventricular (wedge pressure 18-20, pulmonary artery pressure systolic/diastolic/mean 42/20/29; cardiac output 5.9 mL/minute). The laboratory tests that were performed in order to exclude the connective tissue disorders (IgG, IgM, IgA, ANA, p- and c-ANCA, C3, C4, and ENA) throughout his hospitalization were negative. Possible infections from viruses, microbes, Chlamydia, or tuberculosis (polymerase chain reaction [PCR] negative) were also excluded.
Three weeks later after all the above tests had already been performed, the patient underwent a video-assisted thoracoscopy so that we could reach a final diagnosis. The biopsy revealed a very severe fibrotic disorder throughout most of the lung (end-stage lung) (Figure 3). Histomorphologically, the lesion did not resemble some of the typical interstitial lung patterns (usual interstitial pneumonia, non-specific interstitial pneumonia, desquamative interstitial pneumonia, bronchiolitis obliterans organizing pneumonia, acute interstitial pneumonia, respiratory bronchiolitis -interstitial lung disease). At that time there was a lymphocyte-stimulating test performed with all other possible causative drugs (beta blockers, angiotensin-converting-enzyme-inhibitor), but the results were negative showing no clear immunologic trigger mechanism from the chronically taken drugs.
This was a chronic interstitial pneumonitis with eosinophils and fibrosis with a honeycombing image. This process was acute and progressing very rapidly and was directly dependent on the patient's radiographic contrast media exposure. The patient was treated with corticosteroids, receiving a gradually reducing dose (from 60 mg to 25 mg). One year later, in a scheduled check-up visit, his clinical condition was improved (6MWT, lung function parameters [TLC 55% predicted, FEV1 51% predicted]) (Figure 4). Throughout the patient's evaluation period, no peripheral blood eosinophilia was observed.
Axial, high resolution CT images of the lungs in a patient with eosinophilic pulmonary fibrosis demonstrate peripheral distribution of airspace disease and patchy peripheral ground-glass opacities. . Abbreviation: CT, computed tomography.