Alveolar capillary basement membrane lesions in goodpasture's syndrome and idiopathic pulmonary hemosiderosis

An ultrastructural study of lung distinguished between lesions of the alveolar capillary basement membrane in a case of Goodpasture's syndrome and in three cases of idiopathic pulmonary hemosiderosis.In Goodpasture's syndrome, diffuse vascular injury with wide endothelial gaps, diffusely fragmented basement membranes and an electron dense layer on the basement membrane was found.In idiopathic pulmonary hemosiderosis, focal ruptures of the basement membrane were associated with hydropic changes in pneumocytes and, although fibroblasts were not seen, collagen deposition occurred within the basement membrane.Immunofluorescent studies failed to show deposition of immunoglobulins G (IgG), A (IgA), M (IgM) or C₃ in the lung in either disease. The ultrastructural lesions appear to separate these clinically similar entities.     

Nitroglycerin-responsive pulmonary hypertension in idiopathic pulmonary hemosiderosis

Idiopathic pulmonary hemosiderosis (IPH) is an uncommon disease found predominantly in pediatric patients. It can produce severe chronic pulmonary injury that results in chronic hypoxemia, pulmonary insufficiency, and progressive pulmonary fibrosis, leading to irreversible pulmonary hypertension and death. We studied the pulmonary hemodynamics in an 9-yr-old boy with IPH to determine if pulmonary hypertension contributed to exacerbations of this disease. Our results showed that this patient demonstrated pulmonary hypertension during acute exacerbations. Initially, the elevated pulmonary artery pressure responded both to oxygen and to a pulmonary vasodilator in the form of nitroglycerin. However, this improvement was not sustained. We conclude that pulmonary hypertension is probably a result of chronic hypoxemia experienced by patients with pulmonary hemosiderosis. Further investigation is warranted to assess whether or not intervention aimed at reducing pulmonary artery pressure in IPH improves outcome.     

Rheumatoid-like arthritis presenting as idiopathic pulmonary hemosiderosis: a report and review of the literature

Idiopathic pulmonary hemosiderosis, an unusual form of the alveolar hemorrhage syndromes, has been reported in association with several connective tissue disorders. Rheumatoid arthritis (RA), however, rarely presents in this manner. We discuss a young adult with recent onset RA following idiopathic pulmonary hemosiderosis, and review the 3 cases previously reported.     

Life threatening exacerbation in idiopathic pulmonary hemosiderosis salvaged by cyclophosphamide infusion

A seven-year-old girl presented with frequent fever, cough and shortness of breath of three months duration. On the basis of her clinical features, peripheral blood and sputum findings, she was diagnosed as a case of idiopathic pulmonary hemosiderosis. After initial stabilisation with steroids and chloroquine, she presented four years later with massive pulmonary hemorrhage and respiratory failure, which responded dramatically to cyclophosphamide infusion. The rare occurrence of pulmonary hemosiderosis and different treatment regimens is discussed.     

Treatment of life-threatening primary pulmonary hemosiderosis with cyclophosphamide.

This report describes a five-year-old boy with severe pulmonary hemorrhage caused by primary pulmonary hemosiderosis with cow's milk sensitivity. After failing to respond to corticosteroids and azathioprine, he dramatically improved after being given cyclophosphamide. He worsened after discontinuation of cyclophosphamide on two occasions and improved significantly with its reinstitution. Cyclophosphamide was continued for 14 months without further bleeding or adverse effects. The patient has remained in remission for nearly five years. Cyclophosphamide may be a life-saving alternative therapy for refractory pulmonary hemorrhage with pulmonary hemosiderosis.     

Unusual presentation: pulmonary hemosiderosis with celiac disease and retinitis pigmentosa in a child

Idiopathic pulmonary hemosiderosis (IPH) is a rare disease characterized by anemia, hemoptysis and recurrent alveolar hemorrhage. The combination of IPH and celiac disease (CD) is extremely rare. We report a 9-year-old boy with Lane-Hamilton syndrome, co-occurrence of pulmonary hemosiderosis with CD. This presentation is unique presentation because he has also retinal pigmentation.     

Goodpasture's syndrome with normal renal function.

Two male patients with Goodpasture's syndrome manifesting as severe pulmonary hemorrhage with minimal renal abnormalities are described. Both patients had microscopic hematuria with normal renal function, and one had transient proteinuria. Renal glomerular histology was normal and electron microscopic findings revealed no electron-dense deposits, but immunofluorescence of immunoglobulin G (IgG) was positive in a linear fashion along glomerular capillary basement membranes in both patients. Pulmonary hemorrhage was arrested following prednisone therapy, and both patients have normal pulmonary and renal function at five and 13 months of follow-up. The literature on patients with Goodpasture's syndrome, pulmonary hemorrhage and normal renal function with minimal proteinuria is reviewed. It is suggested that a subset of patients with Goodpasture's syndrome have pulmonary hemorrhage as their major manifestation. Since prednisone seems to have an apparent beneficial effect on pulmonary hemorrhage, and relatively good prognosis, this diagnosis should be considered in patients with idiopathic pulmonary hemorrhage.     

A case of idiopathic pulmonary hemosiderosis of adult onset]

A 51-year-old man presenting with hemoptysis was admitted to our hospital. Chest radiography revealed air space consolidation in the right lung field. Laboratory data showed anemia, hypoxemia, and no evidence of inflammatory signs, bleeding tendency, renal dysfunction, or collagen vascular diseases. Tests of anti-GBM antibody, P-ANCA, and C-ANCA were negative. Microscopic examination of the lung tissue specimens obtained by video assisted thoracic surgery revealed hemorrhage and numerous hemosiderin-laden macrophages in the alveoli. No deposition of immunoglobulin and vasculitis were seen. These findings were consistent with a diagnosis of idiopathic pulmonary hemosiderosis. Steroid therapy had a limited effect, and the patient died. Idiopathic pulmonary hemosiderosis of adult onset is rare in Japan.     

Adult patients with idiopathic pulmonary hemosiderosis: a comprehensive review of the literature

Clinical Rheumatology - Idiopathic pulmonary hemosiderosis (IPH) is a rare disease without a known incidence or prevalence in adults. Our knowledge of this entity is limited as there is no...     

Celiac disease and pulmonary hemosiderosis in a patient with chronic granulomatous disease

We report on a patient with the hitherto undescribed combination of chronic granulomatous disease, pulmonary hemosiderosis, and celiac disease. The hemosiderosis resolved with a gluten-free diet and glucocorticosteroid pulse therapy, but the restrictive lung function pattern remained unchanged. Lung function improved markedly by immunosuppression with daily glucocorticosteroid and azathioprine treatment.     

Pulmonary hypertension and beta-thalassemia major: report of a case, its treatment, and a review of the literature

Pulmonary hypertension is a common complication of beta-thalassemia major. We report a case of successful treatment of pulmonary hypertension in a patient with beta-thalassemia major and review the literature on pulmonary hypertension and beta-thalassemia major. A 28-year-old man with beta-thalassemia major, splenectomy, hepatitis C, and hemosiderosis who presented with increasing dyspnea on exertion was diagnosed with pulmonary hypertension. After receiving continuous epoprostenol infusion and desferoxamine, his functional capacity and hemodynamic status improved. To our knowledge, this is the first case of pulmonary hypertension associated with beta-thalassemia treated with continuous epoprostenol infusion and desferoxamine. Epoprostenol, beneficial in the treatment of other types of pulmonary hypertension, may ameliorate the morbidity and mortality of pulmonary hypertension associated with thalassemia.     

Unusual pulmonary findings in mucolipidosis II

We report undescribed pulmonary findings in a child with mucolipidosis II (ML-II). Children with ML-II bear significant pulmonary morbidity that may include extensive pulmonary fibrosis, persistent hemosiderosis as well as pulmonary airway excrescences as they reach preschool age.     

Idiopathic pulmonary hemosiderosis in children. A report on 7 cases (author's transl)

The authors describe 7 cases of idiopathic pulmonary hemosiderosis occurring in children, and emphasize the diagnostic value of the association of pulmonary and anemic manifestations, one of which could appear to be an isolated finding and thus delay diagnosis. Complementary investigations are discussed and the relative inocuity of a lung biopsy with a Gerbeaux needle, and the greater value of bronchoscopic samples over those obtained by gastric intubation for evaluating the presence of siderophagic activity emphasized. Functional pulmonary tests, especially for blood gases and ventilatory function, were also conducted. The difficulties in classifying the various types of pulmonary hemosiderosis, especially the "idiopathic" form in children, are outlined. Immunological disorders must be systematically searched for in order to choose the most effective therapy: corticoids or immunodepressants.     

Hypertensive pulmonary vascular disease in alpha-transposition of the great arteries

In subjects with classic complete transposition (d-transposition) of the great arteries, the pulmonary arterial plexiform lesion, characteristic of chronic high levels of pulmonary arterial pressure, was observed in those with an intact ventricular septum as well as in those with a ventricular septal defect. The lesion was not observed before age 12 months. Among 16 patients aged 12 to 30 months, the plexiform lesion was observed in 6 of 12 patients (50 percent) with an intact ventricular septum and a closed ductus arteriosus and in 1 of 4 patinets with an associated ventricular septal defect. The basis of the plexiform lesion, which is considered a sign of chronic severe pulmonary hypertension, occurring in subjects with an intact ventricular septum and a closed ductus arteriosus is not explained. The phenomenon observed in this study confirms the earlier observations of others.     

Idiopathic Pulmonary Hemosiderosis Associated with Emphysematous Change in an Adult Who Underwent Lung Transplantation

Idiopathic pulmonary hemosiderosis is characterized by repeated alveolar hemorrhaging. We herein report a 52-year-old Japanese woman who had shortness of breath, diffuse small nodules, thin-walled cysts, and bronchiolectasis. A surgical lung biopsy revealed peribronchial hemosiderosis, centrilobular emphysema, and fragile elastic fibers of the alveolar septa and small vessels. She ultimately underwent living-donor lung transplantation five years after the first visit.     

Segmental wall motion abnormalities in an individual with idiopathic pulmonary hemosiderosis

Idiopathic pulmonary hemosiderosis (IPH) is a rare condition characterized by diffuse pulmonary hemorrhage of unknown etiology. Cardiac involvement in the form of myocarditis and right ventricular hypertrophy have been reported to occur in association with IPH, although findings on echocardiography have not been described. Herein is presented a case of an adult with IPH and echocardiographic abnormalities.     

Right ventricular infarction with cardiac rupture in an infant with pulmonary valve atresia with intact ventricular septum

This report describes an 8 day old infant with pulmonary valve atresia, hypoplastic and hypertensive right ventricle and myocardial sinusoid-left anterior descending coronary artery connections. A large right ventricular sinusoid complex developed that was associated with transmural right ventricular necrosis and cardiac rupture. This is the first report to document transmural myocardial infarction and rupture in pulmonary valve atresia with intact septum, and this condition should be added to the causes of myocardial infarction in infancy. A reduced number of caliber of sinusoid-coronary artery channels may be responsible for right ventricular damage in this condition.     

Idiopathic pulmonary hemosiderosis in adults

Idiopathic pulmonary hemosiderosis (IPH) is a rare cause of diffuse alveolar hemorrhage characterized by abnormal deposition of hemosiderin iron in the macrophages of alveoli. Most cases occur in children. In adults, IPH is rare: almost 10 cases reported during the late 10 years. We report the case of a 20-year-old-girl with IPH. There was no evidence of pulmonary vasculitis or capillaritis. Long-term treatment with systemic corticosteroids was followed by clinical remission lasting 4 years.     

Two cases of idiopathic pulmonary hemosiderosis: analysis of chest CT findings]

Chest CT findings are reported in two cases of idiopathic pulmonary hemosiderosis. In both cases, CT was performed after remission of an acute exacerbation following corticosteroid therapy. Case 1 was a 17-year-old woman with Down's syndrome. Chest radiograph showed diffuse ground-glass like and reticulonodular shadows, which were predominant in the bilateral lower lung fields. Chest CT showed a diffuse increase of lung filed density, especially in the dorsal zone of both lower lobes. Open lung biopsy revealed hemorrhage and numerous hemosiderin-laden macrophages in the alveoli, and in addition, marked fibrous thickening of the alveolar septa. Case 2 was a 7-year-old girl. Chest radiograph showed diffuse micronodular shadows in both lungs. Chest CT showed diffuse poorly-circumscribed micronodular lesions with uniform distribution, and lung field density was normal except for the right upper lobe with patchy infiltrates. Although lung biopsy was not performed, fibrous thickening of the alveolar septa was presumed to be mild even if present, since pulmonary function and blood gas analysis were within normal limits. In these two cases, lung field density of CT seemed to reflect the degree of diffuse fibrous thickening of the alveolar septa, and it is suggested that CT is valuable in the evaluation of fibrous thickening of the alveolar septa secondary to recurrent pulmonary hemorrhages. Comprehensive review of CT findings of idiopathic pulmonary hemosiderosis was also performed.     

Atrial level right to left intracardiac shunt associated with postoperative hypoxemia: demonstration with contrast two-dimensional echocardiography

Transient hypoxemia is not uncommon after major cardiac or thoracic surgery. The differential diagnosis includes atelectasis, pulmonary embolus, pneumonitis, congestive heart failure and several other diverse cardiovascular and pulmonary problems. Less well recognized is transient right to left intracardiac shunting through a patient foramen ovale or previously unsuspected atrial septal defect. Three cases of clinically important hypoxemia associated with right to left shunting after aortocoronary bypass surgery are presented. The right to left shunting was documented with contrast-enhanced echocardiography, which is a simple, inexpensive and accurate means of screening patients for intracardiac right to left shunts and may play a valuable role in the postoperative management of patients.