Idiopathic Pulmonary Hemorrhage Research Articles

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 sybdrome 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.

Immune Aspects of the Glomerulonephritis Associated with Idiopathic Pulmonary Hemorrhage.

Excerpt Reports of the syndrome of recurrent pulmonary hemorrhage with glomerulonephritis (Goodpasture's syndrome) have emphasized the fulminant course of the disease. Two cases are presented with ...

Idiopathic pulmonary hemorrhage in pregnancy. Report of a case suggesting early pulmonary hemosiderosis with clinical recovery after steroid therapy.

THE following is a report of a young, seven-month-pregnant woman who was gravely ill with cough, breathlessness, cyanosis, bilateral pulmonary infiltration on x-ray study and a hemoglobin of only 4.8 gm. per 100 ml. Because of the characteristic clinical picture and the absence of any other cause of blood loss, the diagnosis of idiopathic pulmonary hemorrhage or of so-called idiopathic pulmonary hemosiderosis was tentatively made, and appropriate treatment was instituted. Of particular interest is the fact that complete remission rapidly followed the use of steroids in this critically ill patient. A biopsy of the lung was obtained on the tenth . . .

Idiopathic pulmonary hemorrhage with hemosiderosis and microcytic anemia.

The radiologist is in a position to make a significant contribution to the explanation of hemoptysis in some patients if he is aware of the characteristic pattern of idiopathic pulmonary hemorrhage. In Europe the condition has been called the Ceelen-Gellerstedt syndrome; in the United States it has most frequently been described as idiopathic pulmonary hemosiderosis. The first description of the pathologic findings was made by Virchow more than a century ago. Ceelen reported on the autopsies of 2 cases in 1921 (5). Waldenstrom in 1938 correlated the clinical with the pathological picture (12). Recently the reports have been more numerous, and at present approximately 60 cases have been described, most of them with autopsy documentation. According to these reports, the disease usually is fatal within a few years. However, several patients have been reported as being alive several years after the diagnosis was made (2, 7, 9). Case Report M. C. S., a 9-year-old white girl, was admitted to Babies and Childrens Hospital on March 25, 1953, because of expectoration of small amounts of blood-streaked sputum for three or four days. For several months before admission she had been experiencing low-grade fever with frequent respiratory infections, for which she had been treated repeatedly with sulfonamides and antibiotics. A chest roentgenogram made elsewhere two months previously was reported to be normal. The admission rectal temperature was 37.6° C, pulse 120, respirations 24. The patient appeared pale but not acutely ill. The physical examination, including the heart and lungs, was otherwise normal. The blood showed a hemoglobin of 8.6 gm. per cent, a hematocrit of 30 per cent, and 0.4 per cent reticulocytes. The white count was 4,100, with a normal differential, including 2 per cent eosinophils. Other laboratory tests, including sedimentation rate, cold agglutinins, febrile agglutinins, heterophil reaction, tuberculin reactions, bleeding and clotting times, serum proteins, stool guaiac examinations, and routine sputum cultures, gave normal results. No acid-fast bacilli were found in the sputum. The postero-anterior chest roentgenogram showed streaky and mottled fine densities, diffusely distributed throughout the lungs, but with a tendency to coalescence (Fig. 1). During one week in the hospital the course was mildly febrile (maximum temperature 38° C.) and slight hemoptysis continued. One unit of whole blood was given, and the patient was discharged with a hemoglobin of 11.2 gm. per cent and hematocrit of 35 per cent. A few days later the child was readmitted for bronchoscopy and bronchography. Both studies were normal. A postero-anterior roentgenogram obtained nineteen days after the original examination revealed a marked degree of improvement (Fig. 2). A third admission occurred on June 29, 1953, following another episode of hemoptysis and fever.