Pulmonary hemosiderosis (PH) is characterized by repeated episodes of intra- alveolar bleeding that lead to abnormal accumulation of iron as. Idiopathic pulmonary hemosiderosis is a rare disease characterized by repeated episodes of bleeding into the lungs, which can cause anemia and lung disease. Rev Chir Oncol Radiol O R L Oftalmol Stomatol Ser Oftalmol. Jan-Mar;33(1 ) [Corneal hemosiderosis]. [Article in Romanian]. Cernea P, Talea L.
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Idiopathic pulmonary hemorrhage IPH is a rare cause of alveolar hemorrhage AH with unknown etiology that primarily affects children. The process has a variable clinical progression, and its diagnosis is established after excluding all causes of AH. Herein, the authors hemosideroiss a case of IPH in an adult male patient referring cough and hemoptysis.
The conventional radiography computed tomography imaging identified zones of consolidation and areas of ground-glass attenuation in the lower lobes and lingula. Forced spirometry, bronchoscopy, and laboratorial tests yielded normal results.
Several alveolar hemosiderin-laden macrophages were identified on bronchoalveolar lavage and lung biopsy. Thus, the histopathological findings associated with clinical data were compatible with IPH. Neste artigo, os autores relatam um caso de HPI em paciente acalah sexo masculino, adulto, referindo tosse e hemoptise.
Idiopathic pulmonary hemorrhage IPH is a rare cause of diffuse alveolar hemorrhage whose etiology is unknown. The process, which affects primarily children, is characterized by intra-alveolar accumulation of red blood cells and hemosiderin-laden macrophages. The patients present recurrent episodes of pulmonary hemorrhage, which may occasionally determine ventilatory restriction. The clinical presentation is extremely variable, and the most common symptoms include iron deficiency anemia, diffuse pulmonary avalah, and hemoptysis 3, adalab, 10, 11, IPH is a diagnosis of exclusion that is reached when all other causes of pulmonary hemorrhage have been ruled out.
Herein, the authors report a case of IPH in a male patient referring cough and hemoptysis, and review morphological and clinical findings of this uncommon process. Male patient, 45 years old, hairdresser, came to the hospital service hejosiderosis cough and hemoptysis for twelve months.
The medical history included hypertension hemosideross daily use of enalapril 10 mgsleep apnea, and previous lung infection by Haemophilus parainfluenzae. The patient denied dyspnea, chest pain, fever, smoking, frequent contact with asbestos or birds, or allergic processes. Normal pulmonary auscultation was observed at physical examination.
Hemosiderosis – Wikipedia
The other organs and systems also showed no changes. Conventional chest radiography identified the presence of ill-defined opacity in the lower lobes Figure 1. Computed tomography CT scan of the chest revealed the presence of zones of consolidation and patchy areas of ground-glass attenuation in the lower lobes and lingula Figure 2.
Bronchoscopy revealed endobronchial lesions, bloody material, but pathologic secretions on bronchial lumen were not identified. The cytological examination of bronchoalveolar lavage BAL exhibited the presence of several vacuolated macrophages showing brown intracytoplasmic granular pigment.
Idiopathic pulmonary hemorrhage: morphology and differential diagnosis. Case report
Doppler echocardiography examination showed the presence of altered relaxation of the left ventricle with preserved systolic performance. CT scans imaging of the abdomen revealed normal findings. The relevant laboratory tests are described in Table 1. The patient underwent biopsy segmentectomy of the lingula and lower basal segments of the left lung.
The surgical specimen consisted of two cuneiform portions of cortical pulmonary parenchyma, which together weighed On gross examination, red-brownish areas of the lung parenchyma were identified. Microscopy demonstrated numerous vacuolated macrophages containing intracytoplasmic brown granular pigment Figure 3which occupied several alveolar spaces and alveolar ducts and some respiratory bronchioles Figure 4.
Arteries, veins and capillaries also had normal appearance, without evidence of vasculitis Figure 5. On Prussian blue stains, the intracytoplasmic granules of the alveolar macrophages proved to be hemosiderin Figure 6. The histopathological findings related to the clinical data were then compatible with IPH. After a day follow-up, including daily use of prednisone 40 mg, the patient reported a marked reduction in the frequency and intensity of hemoptysis. IPH usually affects children, but in 20 percent of the patients the process is found in adults.
There is no gender-based prevalence in children. The clinical presentation is most often associated with dyspnea, cough, hemoptysis, substernal chest pain, and fatigue due to iron-deficiency anemia. The episodes of pulmonary hemorrhage are recurrent and intermittent. Respiratory distress progressing to respiratory failure is uncommon. Chest examination is non-specific crackles and consolidations. Radiographic findings of IPH consist of patchy or diffuse bilateral areas of airspace consolidation, mostly affecting the perihilar regions and lower lung zones.
Zones of ground-glass attenuation may be visualized on CT scans. On gross examination, the compromised lungs show red, reddish brown, gray, or black, ill-defined areas.
Strong reaction to siderosis is observed on Prussian blue stain. Neutrophilic capillaritis may also be seen, but it tends to be mild. Long-term IPH may lead to interstitial fibrosis. IPH diagnosis is made on the basis of clinical, radiologic, laboratorial and histopathological findings. Final diagnosis can be established only when any evidence of pulmonary vasculitis, nonspecific granulomatous inflammation or diseases associated with deposition of immunoglobulins have been excluded.
The process is associated with a great variety of clinical syndromes, and most patients complain of hemoptysis. Microscopy reveals that DAH commonly presents an accumulation of intra-alveolar red blood cells and hemosiderin-laden macrophages.
Specific classification of DAH syndromes requires correlation with the clinical history, laboratory results, and lung biopsy findings.
The differential diagnosis of DAH may be classified immunologically by the immunofluorescence or electron microscopic findings or histologically by the data on lung biopsy.
Alveolar hemorrhage AH may be often associated with an organizing pneumonia consisting of intra-alveolar plugs of loose, organizing fibroblastic connective tissue reminiscent of the bronchiolitis obliterans organizing pneumonia BOOP pattern 5, 7, 9, 15, 25, The major pathologic features of WG are vasculitis, parenchymal necrosis, granulomatous inflammation mixed inflammatory infiltrateand alveolar hemorrhage in some cases.
In active generalized WG, positive staining for C-ANCA in neutrophil cytoplasm on immunofluorescence analysis is found in 84 to 99 percent of patients.
Vasculitis of WG may affect arteries, veins, or capillaries in the form of capillaritis 6, 9, 13, 19, 24, It is mediated by circulating cytotoxic antibody generally immunoglobulin class G [IgG] that reacts with pulmonary and glomerular basement membrane. Evidence of this anti-basement membrane antibody in the serum, kidney or lung biopsies is required for the diagnosis of GS These authors suggested that the use of products related to permanent waving could be linked with the pulmonary disease.
Moreover, the histopathological findings of the sample did not demonstrate the presence of pulmonary vascular lesions or inflammatory process affecting periseptal alveolar capillaries. The authors of the present report also suggest the hypothesis, similarly to Bernis et al. Churg-Strauss syndrome CHS is characterized by the presence of asthma, peripheral blood eosinophilia, neuropathy, radiographic pulmonary infiltrates, paranasal sinus abnormalities, and vasculitis.
Presence of diffuse pulmonary hemorrhage and capillaritis may be seen 24, Microscopic polyangiitis MP is a necrotizing vasculitis with few or no immune deposits that involves small vessels, showing necrotizing arteritis of small and medium sized arteries in some occasional cases. The most common lung biopsy findings in MP are pulmonary hemorrhage and neutrophilic capillaritis.
On high magnification, thickening of the alveolar walls with many neutrophils on the septal interstitium can be found. The neutrophils may show karyorrhexis and fill in the surrounding alveolar spaces 7, 9, 14, 15, In congestive pulmonary vasculopathy, the compromised lungs show extensive and severe fibrosis of the elastic arteries.
The surrounding lung tissue shows hemosiderosis Pulmonary siderosis PS is defined as the deposition of iron or iron oxides in the lungs after inhalation exposure. The process is more commonly found among occupational exposures. PS occurs predominantly in mining hematite, limonite, siderite, and emerysteel mills, foundries, boiler scaling, welding, and in the grinding, polishing, and refining of metal alloys.
Pure exposure to iron is rarely associated with clinically significant disease unless other dusts are also present, mainly silica. Microscopy reveals numerous iron-filled macrophages involving respiratory bronchioles and alveolar ducts 20, 22, Airway disease related to mineral dust and macrophages associated with fibrosis of alveolar ducts can also be identified.
The presence of silica or asbestos mixed pneumoconiosis modifies the pathologic findings according to the degree of this deposition. In the present report, the authors reported a case of IPH in an adult male patient referring cough and hemoptysis, after exhaustive clinical, laboratorial and radiologic investigation to exclude other possible causes of AH.
Table 2 describes similar cases of IPH found in the international literature and comparable to the present report in hemosiderosos to undetermined etiology, clinical course, and treatment. The most frequent cause of death in IPH is related to acute respiratory failure secondary to massive hemorrhage.
Chronic respiratory failure and cor pulmonale are related to the heemosiderosis of severe fibrosis. Hmosiderosis patients develop repeated episodes of hemoptysis, or persistent dyspnea and anemia 5, 11, 21, The use of corticosteroids must be considered, but the response is variable 1, 3, 8, A good prognosis is hemosidefosis in this case, although the possibility of short episodes of recurrence must be considered.
Management of a parturient with an acute exacerbation of idiopathic pulmonary haemosiderosis and posterior spinal instrumentation. Br J Anaesthv. Remission of Goodpasture’s syndrome after withdrawal of an unusual toxic.
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