Goodpasture Syndrome
Published: 28 March 2022
Published: 28 March 2022
Goodpasture syndrome is a rare but potentially deadly disorder that affects about 0.5 to 1.8 people per 1,000,000 every year (DeVrieze & Hurley 2021).
Recognising the symptoms of Goodpasture syndrome and escalating care immediately is crucial in preventing fatality (DeVrieze & Hurley 2021).
Goodpasture syndrome, which may also be referred to as anti-glomerular basement membrane (anti-GBM) disease, is a potentially life-threatening autoimmune disorder where the body produces antibodies that attack the lining of the lungs and kidneys (DeVrieze & Hurley 2021; NKF 2015).
The disorder is characterised by:
(GARD 2017; NKF 2015; Lee 2021)
Without prompt treatment, symptoms can progress rapidly, leading to life-threatening bleeding in the lungs, renal failure and potentially death (Story & Marcin 2018; NKF 2015).
It’s unknown exactly what causes anti-GBM antibodies to develop, but they’re thought to be triggered by an environmental insult in a person with a pre-existing genetic susceptibility (GARD 2017).
Once anti-GBM antibodies are produced, they attack the collagen present in the alveoli and capillaries in the lungs, and/or the glomerulus (filtering units in the kidneys), causing bleeding in the lungs and inflammation in the kidneys (GARD 2017).
Potential environmental triggers include:
(DeVrieze & Hurley 2021; NKF 2021)
Those who have a genetic predisposition to the creation of anti-GBM antibodies appear to have specific human leukocyte antigen (HLA) subtypes (DeVrieze & Hurley 2021).
Those who appear to be at increased risk of Goodpasture syndrome include:
(DeVrieze & Hurley 2021)
Lung-related symptoms | Kidney-related symptoms |
---|---|
|
|
(GARD 2017)
A diagnosis is made upon the presentation of:
(GARD 2017; NKF 2015; Lee 2021)
Bleeding in the lungs should be diagnosed and treated the most urgently, as this is the main cause of early death in patients with Goodpasture syndrome (GARD 2017).
There are several differential diagnoses that may need to be ruled out, including:
(GARD 2017)
Patients will often present critically ill and will require immediate haemodialysis, along with intubation in the case of respiratory failure. At this stage, a kidney biopsy may also be taken to confirm the diagnosis (DeVrieze & Hurley 2021).
The primary goals of treatment are to:
(GARD 2017; DeVrieze & Hurley 2021; Heitz & Falck 2018)
Patients may also require supportive care such as oxygen administration, mechanical ventilation and/or blood transfusion (Lee 2021).
Even with appropriate treatment, renal failure is common, in which case the patient will require dialysis and in severe cases, a kidney transplant. However, more than 70% of surviving patients will only require dialysis temporarily (DeVrieze & Hurley 2021).
Post-discharge, the patient will need to continue treatment. Plasmapheresis is generally performed daily until anti-GBM antibodies are undetectable, and immunosuppressive therapy will continue for a further three to six months after cessation of plasmapheresis. Renal function will also be monitored long-term (DeVrieze & Hurley 2021; GARD 2017).
Overall, the five-year survival rate of Goodpasture syndrome is over 80%, however, it’s crucial that treatment is commenced immediately, as delays may lead to fatality (DeVrieze & Hurley 2021).
Question 1 of 3
What is believed to be the cause of Goodpasture syndrome?