Failure to diagnose Churg-Strauss Syndrome

Presentation

A 44-year-old man came to the ED with leg swelling and ecchymosis present for the past six days. He denied nausea, vomiting, or diarrhea.

The patient reported a history of chronic bronchial asthma. He had been seen by his internal medicine physician — IM Physician A — 12 days earlier for a suspected urinary tract infection. The patient began taking ciprofloxacin and developed leg swelling after six days. He returned to IM Physician A who prescribed furosemide. The patient's symptoms did not improve, which prompted the visit to the ED.

Labs revealed an elevated white blood cell count, elevated liver enzymes, and proteinuria. The results of a chest x-ray and renal ultrasound were normal. The ED physician recommended hospital admission.

Physician action

The patient was admitted to the care of IM Physician B  (the patient’s internist did not have hospital privileges). IM Physician B's differential diagnoses included sepsis, etiology unclear; bronchitis; asthma; fever; hyponatrema; elevated liver enzymes; and urinary tract infection. He requested nephrology and infectious diseases consultations.

Multiple tests were ordered, including a skin biopsy that demonstrated vasculitis of unknown etiology. The patient was treated with IV methylprednisolone and seemed to improve. He was discharged after six days in the hospital with a prescription for onprednisone, 40 mg per day for seven days. He was instructed to follow up with IM Physician B.

The patient saw IM Physician B three times in the week after discharge. The patient reported improvement, but still had respiratory complaints, abdominal pain, a rash, and “on and off fatigue.”

At the second visit, a tapering dose of prednisone was ordered, 20 mg to 5 mg over four days. On the third visit, the patient was referred to a pulmonologist and a rheumatologist. He was also asked to return to the IM Physician B's office in one week. The patient did not follow up with the referrals and transferred his care back to IM Physician A.

Approximately six weeks after discharge, the patient returned to the hospital with shortness of breath, hemoptysis, and weakness that had progressed to his arms. He now had difficulty standing or grasping objects.

The IM Physician A, now with hospital privileges, saw the patient. His admission notes reported that the patient had been seen as an outpatient after the prior admission, but had been lost to follow up. The patient underwent nerve and muscle biopsies that resulted in a diagnosis of vasculitis secondary to Churg-Strauss Syndrome (CSS).

Over the next year, the patient’s condition deteriorated. He became weaker and could not walk. He was hospitalized on several occasions for renal insufficiency and thromboembolic disease. The patient committed suicide.
 

Allegations

The patient’s family filed a lawsuit against IM Physician A and B. The allegations included failure to timely diagnose and treat the patient’s CSS. They further alleged that these physicians should have ensured that the patient would be followed by a rheumatologist. As a result, the patient became unable to walk independently, take care of his personal needs, or work which led to severe mental anguish and suicide.
 

Legal implications

Independent experts who reviewed this case agreed that CSS is rare, but that an internal medicine physician should have been aware that treatment requires long-term use of high dose prednisone. The experts agreed that IM Physician B failed to maintain the patient on an adequate prednisone regimen.

Further, there was an issue with the production of the EHR in the lawsuit that gave the perception that CSS had been diagnosed but not addressed much earlier in the patient’s course of treatment.
 

Disposition

This case was settled on behalf of both physicians.
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