TESTING: Kidney, lung damage
Over 5 days, Mr. C’s intermittent low-grade fevers continue. Laboratory tests are negative for HIV antibody, hepatitis panel, and antinuclear antibodies (ANA). C-reactive protein is elevated at 27.8 mg/dL (normal range,
Renal ultrasound is normal, but preliminary renal biopsy shows rapidly progressive glomerulonephritis. The internist immediately starts dialysis, cyclophosphamide at 1.5 mg/kg, and prednisone, 1 mg/kg. The pathology report on the renal biopsy describes extensive crescentic glomerular destruction, with inflammatory cells present.
Ten days after admission, Mr. C develops hemoptysis, and chest radiography shows increasing alveolar infiltrates. The attending internist consults pulmonary and critical care services.
The consultant suspects a pulmonary-renal syndrome because of bilateral alveolar infiltrates (diffuse alveolar hemorrhage). The internal medicine team continues high-dose corticosteroids, followed by plasmapheresis.
Brain MRI shows subacute to chronic infarcts involving the right basal ganglia and corona radiate and mild to moderate small vessel ischemic changes. Old areas of hemorrhage are noted within both cerebellar lobes, left temporal lobe, right basal ganglia, right parietal lobe, and right frontal lobe.
During follow-up interviews, Mr. C often cannot recall recent dialysis or plasmapheresis and reports no physical symptoms. His short-term memory continues to deteriorate; he would forget to eat if not cued by family or nursing staff. He shows global cognitive deficits and is increasingly withdrawn and flat.
The authors’ observations
Although few case reports have associated Goodpasture’s syndrome with neurobehavioral changes, the apparent relationship of Mr. C’s medical symptoms with the worsening of his cognitive impairment suggests a link.
Mr. C’s MRI findings also might suggest CNS vasculitis, which affects small arteries of the cerebral and spinal cord leptomeninges and parenchyma, leading to CNS dys-function.6-8 CNS vasculitis can result from primary nervous system involvement or from a secondary systemic process such as Goodpasture’s syndrome.9
We rule out lupus because Mr. C is ANA-negative; this test has 99% sensitivity for lupus.10
Goodpasture’s syndrome: A rare autoimmune disease
Goodpasture’s syndrome, which afflicts 1 Patients typically present with alveolar bleeding, rapidly progressive acute renal failure with proteinuria,1 and pulmonary symptoms such as dyspnea and hemoptysis.2
Possible triggers include:
- viral upper respiratory tract infection (20% to 60% of patients)3
- exposure to hydrocarbon solvents (3,4
Mr. C was exposed to solvents during the 15 years he worked in a factory. Some researchers believe a noxious event among genetically susceptible persons damages basement membrane and exposes an antigen that triggers IgG auto-antibody production.3,4
Malaise, weight loss, and fever are atypical in Goodpasture’s syndrome but could suggest concomitant vasculitis.5
OUTCOME: Ongoing disability
Mr. C is hospitalized for 6 weeks. He receives cyclophosphamide, prednisone, and 10 sessions of plasmapheresis. We prescribe mirtazapine, 15 mg at bedtime, to treat mood symptoms. We chose mirtazapine because of the drug’s sleep-restoring and appetite-stimulating properties.
Mr. C’s fever resolves and pulmonary function soon improves, but his cognitive impairment persists. He has difficulties preparing meals, taking medications, and managing his money.
Mr. C is discharged with a referral to a psychiatrist. He continues taking mirtazapine and a lower dose of prednisone. He requires ongoing hemodialysis and assistance with activities of daily living.
The authors’ observations
Prompt multidisciplinary intervention is critical when patients present with concurrent cognitive and medical symptoms. A thorough psychiatric evaluation can help piece together the illness’ course. The psychiatrist’s role in a multidisciplinary assessment is to:
- document neurocognitive changes
- verify them through collateral information
- correlate these changes with the timing of medical symptoms.
An underlying psychiatric condition can complicate the diagnosis. In these cases, careful interviewing and collateral information can help you discern the chronology of events.