Find information on thousands of medical conditions and prescription drugs.

Goodpasture's syndrome

Goodpasture’s syndrome (also known as Goodpasture’s disease and anti-glomerular basement membrane disease or anti-GBM disease) was first described by Ernest Goodpasture in 1919. It is an rare condition characterised by rapid destruction of the kidneys and haemorrhaging of the lungs. Although many diseases can present with these symptoms, the name Goodpasture’s syndrome is usually reserved for the autoimmune disease produced when the patient’s immune system attacks cells presenting the Goodpasture antigen, which are found in the kidney and lung, causing damage to these organs. more...

Gardner's syndrome
Gastric Dumping Syndrome
Gastroesophageal reflux
Gaucher Disease
Gaucher's disease
Gelineau disease
Genu varum
Geographic tongue
Gerstmann syndrome
Gestational trophoblastic...
Giant axonal neuropathy
Giant cell arteritis
Gilbert's syndrome
Gilles de la Tourette's...
Gitelman syndrome
Glanzmann thrombasthenia
Glioblastoma multiforme
Glucose 6 phosphate...
Glycogen storage disease
Glycogen storage disease...
Glycogen storage disease...
Glycogenosis type IV
Goldenhar syndrome
Goodpasture's syndrome
Graft versus host disease
Graves' disease
Great vessels transposition
Growth hormone deficiency
Guillain-Barré syndrome

Signs and symptoms

Most patients present with both lung and kidney disease, however, some patients present with one of these diseases alone. The first lung symptoms usually develop days to months before kidney damage is evident.

Lung disease

Lung damage may cause nothing more serious than a dry cough and minor breathlessness and such mild symptoms may last for many years before more severe ones develop. At its most serious, however, lung damage may cause severe impairment of oxygenation so that intensive care is required. Deterioration between the two extremes may occur very rapidly, often at the same time as rapid deterioration in the kidney. The patient often does not seek medical attention until he or she begins coughing up blood. The patient may be anaemic due to loss of blood through lung haemorrhaging over a long period. In Goodpasture’s syndrome, unlike many other conditions that cause similar symptoms, lung haemorrhaging most often occurs in smokers and those with damage from lung infection or exposure to fumes.

Kidney disease

The kidney disease mostly affects the glomeruli causing a form of nephritis. It is usually not detected until a rapid advance of the disease occurs so that kidney function can be completely lost in a matter of days. Blood leaks into the urine causing haematuria, the volume urinated decreases and urea and other products usually excreted by the kidney are retained and build up in the blood. This is acute renal failure. Renal failure does not cause symptoms until more than 80% of kidney function has been lost. Symptoms include loss of appetite and sickness at first and then, when the damage is more advanced, breathlessness, high blood pressure and oedema (swelling caused by fluid retention).


Because of the vagueness of early symptoms and rapid progression of the disease, diagnosis is often not reached until very late in the course of the disease. Kidney biopsy is often the fastest way to secure the diagnosis and gain information about the extent of the disease and likely effect of treatment. Tests for anti-GBM antibodies may also be useful, combined with tests for antibodies to neutrophil cytoplasmic antigens, which are also directed against the patient’s own proteins.


As with many autoimmune conditions, the precise cause of Goodpasture’s Syndrome is not yet known. It is believed to be a type II hypersensitivity reaction to Goodpasture’s antigens on the cells of the glomeruli of the kidneys and the pulmonary alveoli, whereby the immune system wrongly recognises these cells as foreign and attacks and destroys them, as it would an invading pathogen.


[List your site here Free!]

Goodpasture's syndrome: unusual presentation after exposure to hard metal dust
From CHEST, 3/1/93 by Peter Lechleitner

An unusual case of Goodpasture's syndrome in a 26-year-old man with occupational exposure to hard metal dust is described. The patient developed a life-threatening interstitial lung disease that was followed by a rapidly progressive glomerulonephritis two months later. To our knowledge, association of Goodpasture's syndrome and hard metal exposure has not been reported previously.

Goodpasture's syndrome is characterized by pulmonary hemorrhage and rapidly progressive glomerulonephritis. It has been observed after exposure to various inhalants, mostly hydrocarbons.[1]

We describe the case of a patient exposed to hard metal dust, in whom antiglomerular basement membrane antibody-mediated Goodpasture's syndrome developed. Hard metal is an alloy of inert tungsten carbide and cobalt, which is considered the causative agent in hard metal lung disease.[2]


A 26-year-old man of previous good health was referred to our hospital because of fever and increasing dyspnea. The last three years before hospital admission, he had been working in a metallurgical plant processing high-melting alloys, mostly hard metal. Laboratory tests showed a marked leukocytosis of 27.500/[mu]l, mild elevation of liver enzyme values, and a microhematuria. The complement fraction C4 was significantly lowered to 0.06 g/L, C3 was in the normal range. The patient's chest roentgenogram showed a barely visible bilateral micronodular pattern that contrasted with the massive compromise of respiratory function.

Treatment with high-dose steroids, imipenem, and erythromycin led to quick improvement of respiratory function. Bronchoalveolar lavage (BAL) was performed and revealed a massive increase in neutrophils, but no pathogenic microorganisms were identified, and cultures from BAL fluid remained sterile. Repeated BAL 12 days later revealed an unremarkable cell pattern and normal lymphocyte subpopulations. Many alveolar macrophages contained both iron-positive and iron-negative, coarse granular inclusions. Scanning electron microscopy, including energy dispersive x-ray spectroscopy, showed high amounts of tungsten in these dust granules, whereas cobalt could not be identified (Fig. 1). BAL fluid examined by atomic absorption spectroscopy was negative for cobalt. A transbronchial biopsy specimen showed a slightly increased desquamation of pigment-laden alveolar macrophages in otherwise normal lung parenchyma.

The patient continued receiving low-dose steroid therapy and remained in stable health for the next two months and did not return to his workplace. Macrohematuria preceded by occasional hemoptyses prompted readmission to the hospital. The chest roentgenogram was unremarkable, and laboratory tests revealed a creatinine concentration of 262.5 [mu]mol.L (2.97 mg/dl). A kidney biopsy was performed and showed extracapillary and intracapillary glomerulonephritis with linear deposits of IgG and complement C3 along the glomerular basement membrane (GBM). A diagnosis of rapidly progressive glomerulonephritis was made and confirmed by the detection of anti-GBM antibodies of the IgG subclass. C4 was markedly reduced to 0.054 g/L (control value, 0.095 g/L). The phenotyping of complement fraction C4 revealed a heterozygous C4A deficiency (C4A3, C4AQ0, C4B1, C4B2). Plasmapheresis and immunosuppressive therapy with cyclophosphamide and steroids led to prompt recovery, and the patient's renal and pulmonary function remained stable during the following 12 months.


Occupational exposure to hard metal can lead to a wide spectrum of pulmonary diseases, including occupational asthma, desquamative or giant cell interstitial pneumonitis, and diffuse interstitial fibrosis.[2] The demonstration of tungsten particles in the lung can be taken as evidence for exposure to hard metal dust.[3] Tungsten carbide itself is probably inert, and cobalt is regarded as the causative agent in hard metal lung disease.[2] Due to its high solubility in body fluids, it is found only infrequently in clinical specimens. In our patient's disease, cobalt might have played a pathogenetic role through various mechanisms of action. On one hand, it might interfere with normal immunoregulation. The induction of a polyclonal B-cell activation has been demonstrated for the metal salt mercuric chloride, causing anti-GBM disease in rats.[4] Alternatively, hard metal dust-induced tissue damage might expose pulmonary basement membrane antigens, with subsequent antibody formation, or allow the binding of otherwise sequestered anti-GBM antibodies in the lung. This mode of action has been demonstrated for hydrocarbons in experimental anti-basement membrane disease of the lung.[5]

Although serologic and immunohistologic findings were clearly diagnostic of Goodpasture's syndrome, the clinical presentation in our patient was atypical. A reason for this might have been the patient's heterozygous C4A deficiency with very low C4 levels not expected in a patient with this phenotype. The binding of complement along the basement membrane and its activation via the classic pathway probably is of importance for the development of parenchymal damage in Goodpasture's syndrome. In contrast to C4B, which preferentially binds to cell surfaces, C4A has higher affinities for immune complexes and antibodies.[6] Thus, the C4A deficiency, resulting in reduced C4 binding along the GBM, might have attenuated the course of glomerulonephritis.

Although the causative role of hard metal exposure for the development of Goodpasture's syndrome in our patient cannot be proved conclusively, the case illustrates the potential complex interrelations among autoimmune disease, immune defects, and exposure to substances with possible antigenic properties.


[1] Beirne GJ, Brennan JT. Glomerulonephritis associated with exposure to hydrocarbons mediated by antibodies to glomerular basement membrane. Arch Environ Health 1972; 25:365-69

[2] Sjogren I, Hillerdal G, Anderson A, Zetterstrom O. Hard metal lung disease: importance of cobalt in coolants. Thorax 1980; 35:653-59

[3] Davidson AG, Haslam PL, Corrin B, Coutts II, Dewar A, Riding WD, et al. Interstitial lung disease and asthma in hard-metal workers: bronchoalveolar lavage, ultrastructural, and analytical findings and results of bronchial provocation tests. Thorax 1983; 38:119-28

[4] Sapin C, Druet E, Druet P. Induction of anti-glomerular basement membrane antibodies in the Brown-Norway rat by mercuric chloride. Clin Exp Immunol 1977; 28:173-79

[5] Yamamoto T, Wilson CB. Binding of anti-basement membrane antibody to alveolar basement membrane after intratracheal gasoline installation in rabbits. Am J Pathol 1987; 126:497-505

[6] Law SKA, Dodds AW, Porter RR. A comparison of the properties of two classes, C4A and C4B, of the human complement component C4. EMBO J 1984; 3:1819-23

COPYRIGHT 1993 American College of Chest Physicians
COPYRIGHT 2004 Gale Group

Return to Goodpasture's syndrome
Home Contact Resources Exchange Links ebay