Progressive massive fibrosis is a recognized complication of pneumoconiosis secondary to pulmonary fibrosis.

Progressive massive fibrosis is a recognized complication of pneumoconiosis secondary to pulmonary fibrosis, with retraction forming conglomerate masses symmetrically in the upper lobes. Idiopathic pulmonary hemosiderosis is known to cause pulmonary fibrosis secondary to returning alveolar hemorrhage with deposition of hemosiderin in the interstitium. We quick in emergencies a case in which progressive massive fibrosis cause to growed in a patient with long-standing idiopathic pulmonary hemosiderosis.

Progressive massive fibrosis has been recognized as a proces occurring in long-standing and cruel silicosis when progressive cicatricial retraction of silicotic masses comes in benign, upper lobe masses. We near a case of progressive massive fibrosis resulting from idiopathic pulmonary hemosiderosis.

CASE REPORT

A 53-year-old woman with a history of idiopathic pulmonary hemosiderosis was admitted to the hospital for evaluation of shortness of breath, pulmonary fibrosis, and nausea. The patient's history actually began 23 years prior to this hospital admission (initially reported by the agency of Sultan et al[1]), when a chaste iron deficiency anemia developed in the third trimester of her third pregnancy. Her hemoglobin continued to fall postpartum despite oral and parenteral iron supplementation. Six month postpartum the patient had disentanglement of diffuse alveolar hemorrhage and hemoptysis. Laboratory evaluation revealed an iron deficiency anemia (hematocrit = 30 percent serum iron = 50 [MICROG]/dl, total iron-binding capacity = 340 [MICROG]/dl), normal urinalysis, and normal serum urea nitrogen even Bronchoscopy revealed blood in the right middle lobe bronchus while a bronchogram was normal. Several more episodes of hemoptysis occurr athwart the next few months and repeated bronchoscopy revealed life-current in the right upper lobe bronchus. make open lung biopsy specimen revealed hemosiderin-laden macrophages and interstitium with no vasculitis. The patient was diagnosed as having idiopathic pulmonary hemosiderosis and was started upon a regimen of high-dose steroids that was slowly tapered to 5 mg orally each day. She did well do not include for occasional episodes of thirsty cough, shortness of breath, and hemoptysis that always resolv with a steroid boost

At the time of this hospital admission the patient complained of progressive dyspnea in succession exertion and a persistent cough although she denied sputum production, febrile disease or hemoptysis. Her medical history and family history were not contributory and she specifically denied any history of silica outlook or cigarette smoking. The admission chest radiograph is shown in Figure 1 Pulmonary function testing revealed an [FEVsub1] and FVC that were 39 percent and 66 percent of predicted, respectively. Thoracic gas contortion was 92 percent of predicted, residual turn was 129 percent of predicted, and diffusing capacity of CO/alveolar ventilation (Dco/Va) was 37 percent of predicted. There was no bronchodilator rejoinder Room air arterial life-current gas analysis revealed a pH of 745 [PaCO.sub.2] of 32 mm Hg and [PaO.sub.2] of 42 mm Hg Other laboratory exhibitions included the following: normal [[alpha].sub.1]-antitrypsin (263 mg/dl); normal serum urea nitrogen and creatinine (11 and 09 mg/dl respectively); normal urinalysis; normal sedimentation rate; and negative antinuclear antibody; rheumatoid factor, and serum antiglomerular antibodies. Fiberoptic bronchoscopy with bronchial wash plus brushing and endobronchial biopsy specimens raise no evidence of acid-fast organisms, fungi, or malignancy, although hemosiderin-laden macrophages were detected

High-resolution comput tomographic (CT) scan of the thorax was performed using 1.5-mm-thick sections each 10 mm from the apices end the bases of the lung during suspended maximum inspiration while in the supine state Mediastinal windows were obtained using a soft-tissue algorithm. Lung windows were obtained with targeted raw data reconstructions of each lung using a bone algorithm. A magnetic resonance (MR) imaging consideration of the thorax was performed using 10-mm contiguous sections in axial and coronal planes via gated techniques for relative T1- and T2-weighted images.

From the CT sections in the axial plane, it was noted that the central masses are extremely dense (Fig 2). The density within the masses forward CT was 361.9 Hounsfield units compared with cancellous bone and cortical bone measurements of 816 and 6093 respectively. Measurements from fibrous malleable tissue in the lateral chest and contrast opacified aorta were 424 and 762 Hounsfield units, respectively.

DISCUSSION

This unusual case was reported greatly earlier in her course by means of Sultan et al.[1] We now quick in emergencies her long-term follow-up as a case of progressive massive fibrosis resulting from idiopathic pulmonary hemosiderosis. We are not aware that this has been previously described.

The clinical manifestations of diffuse alveolar hemorrhage typically include hemoptysis, infiltrates forward the chest radiograph, dyspnea, and variable anemia.[2] All of these characteristics were demonstrated at our patient during her defered course. Diffuse alveolar hemorrhage frequently results from antibasement membrane antibody disease and is therefore associated with glomerulonephritis.[2] However, our patient had normal renal function and urinalyses from end to end her course and serum antiglomerular antibodies were negative. She also had no evidence of systemic vasculitis or collagen-vascular disease, other conditions associated with diffuse alveolar hemorrhage.[3] Finally, she had no known exposing to exogenous triggers such as D-penicillamine or trimellitic anhydride.[3] This patient's clinical course and make open lung biopsy specimens (hemosiderin-laden macrophages and interstitium) are therefore principally consistent with the diagnosis of idiopathic pulmonary hemosiderosis.

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