It's the Presentation, Stupid: A Patient Is Not a Lab Test
It's the Presentation, Stupid: A Patient Is Not a Lab Test
On repeat blood testing, the patient had a 4+ positive ANA titer and 4+ antibodies to double-stranded DNA with low C3 and C4 complement, all consistent with the clinical picture of systemic lupus erythematosus. The patient was placed on 24 mg of oral methylprednisolone twice daily, along with a proton pump inhibitor. All signs and symptoms of active inflammation disappeared completely and rapidly.
The patient was seen by a hepatologist, who thought that azathioprine should be added to the regimen in view of his autoimmune hepatitis. After a normal thiopurine methyltransferase test, the patient was started on azathioprine, aiming toward a daily dose of 2 mg/kg.
The patient's methylprednisolone regimen was successfully tapered a single 16-mg dose daily without a flare in his illness.
Of note, the patient's testicles became atrophic after his bout of orchitis. He recently married and is now concerned that he may be sterile.
Follow-up
On repeat blood testing, the patient had a 4+ positive ANA titer and 4+ antibodies to double-stranded DNA with low C3 and C4 complement, all consistent with the clinical picture of systemic lupus erythematosus. The patient was placed on 24 mg of oral methylprednisolone twice daily, along with a proton pump inhibitor. All signs and symptoms of active inflammation disappeared completely and rapidly.
The patient was seen by a hepatologist, who thought that azathioprine should be added to the regimen in view of his autoimmune hepatitis. After a normal thiopurine methyltransferase test, the patient was started on azathioprine, aiming toward a daily dose of 2 mg/kg.
The patient's methylprednisolone regimen was successfully tapered a single 16-mg dose daily without a flare in his illness.
Of note, the patient's testicles became atrophic after his bout of orchitis. He recently married and is now concerned that he may be sterile.
Take-Home Points
Although laboratory tests can be helpful in supporting one diagnosis or another, the clinical picture trumps any test result. The clinical presentation is the most powerful "test" or "biomarker" available.
A sentinel, powerful clinical manifestation, such as orchitis, is most commonly seen in polyarteritis nodosa. However, orchitis was only a part of this patient's systemic autoimmune disorder that focused on the pericardium, pleura, liver, and testes.
The decision not to treat this patient with steroids between November 2011 and April 2012 because of a negative ANA titer and an "unclear" diagnosis was unfortunate and dangerous because, after infection and cancer were ruled out, systemic lupus erythematosus was the only disease that could explain all of his clinical problems. If steroid therapy had been instituted earlier, perhaps the patient would not have needed pericardial stripping; would not have developed testicular atrophy; and would not have developed further, progressive liver disease. Although institution of steroids can be fraught with potential problems, in this situation that was so clear, not starting steroids resulted in irreversible tissue damage.
You need to "strike while the iron is hot," because there is an optimal therapeutic window of opportunity in every clinical situation.
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