The patient, a 55-year-old woman, went to the emergency room around 11 am on 6/17/22 complaining of dyspnea, cough with blood-tinged sputum, and fever. Her oxygen saturation in the ED was 89%, and she was placed on a non-rebreather mask. She was restless and tachycardic, with accessory muscle usage and bilateral rhonchi. The patient deteriorated rapidly and was intubated while still in the ED. She was also found to be in acute kidney failure, with creatinine of 4.58 and eGFR of 9.9. She was admitted to the ICU, and the admitting critical care doctor felt she had pulmonary renal syndrome, with autoimmune vasculitis on the differential list. He ordered 1 g of IV SoluMedrol, which was given that evening. At around 3:45 p.m., he signed an order for "ANCA screen with reflex to titer," with priority "today." For some reason, that order was canceled immediately. Just after 5 pm, the consulting nephrologist entered a new order for "ANCA Screen (MPOA/PRT3A w/ reflex to ANCA titer." That order was "Cancelled by performing department," at an unknown time.
The next day, 6/18, the admitting doctor's progress note plan included "follow up on cultures and serologies." He ordered two more daily doses of 1 gram of IV SoluMedrol, which were administered that day and the next.
On 6/19, the HPI section of his note said, "Serologies for pulmonary renal vasculitis were ordered," and the plan again included "Follow up on cultures and serologies." On 6/19, at 8:23 p.m., the admitting entered another order for "Anca screen (MPOA/PRT3A) w/ reflex to ANCA titer," with a priority of "routine AM - floor to collect, scheduled time: 6/20/2022 0500." Accordingly, the labs were collected the morning of 6/20 and sent to an outside laboratory, which received them on 6/21.
Also on 6/21, a different critical care specialist requested a rheumatology consult "for possible autoimmune disorder." The rheumatologist saw the patient the same day, and noted the following: “I would reconsider not giving immunosuppressive medications at this point despite not having the available autoimmune antibody results. I am going to go ahead and start IV Solu-Medrol at least 60 mg twice a day….It would be nice to see the results of the ANCA, so we can take a final decision on whether to do immunosuppressive medications like rituximab or Cytoxan alongside the steroids.” The rheumatologist ordered 60 mg of IV push SoluMedrol to be given every 12 hours.
The afternoon of 6/25, preliminary results from the ANCA labs came back positive (with a Proteinase-3 antibody level of 14.2, ref. range <1.0), and the patient was started on plasmapheresis treatments and rituximab. She spent a total of 21 days on the ventilator, but was eventually discharged to a long-term acute care hospital on August 9, and later home. She is nearly weaned off supplemental oxygen and her eGFR at last check was 49.
Files:
Q: Why were the providers not aware of the ANCA cancellation? Was there any notification in the lab section? Was a call placed? to whom?
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Q: Why was the ANCA order cancelled?
A: —
Q: Did the patient's symptoms worsen during the first three days of hospitalization? Especially, did the renal function deteriorate and she develop worsening hemoptysis, hypoxemia, and pulmonary infiltrates?
A: —
Do you believe there might have been medical error?
Appropriate empirical treatment with i.v. Steroids were given to the patient while awaiting ANCA serologies.
Do you believe there might have been causation (i.e. the medical error resulted in an injury)?
I do not see injury stemming from a lack of therapy. The ANCA vasculitis had caused lung and renal injuries. However, these recovered based on the story provided. The providers started immunosuppressive therapy with steroids empirically before knowing ANCA serologies.
What makes you a good expert for this case?
Critical care specialist with >10 years of experience. Vascular neurologist used to treat vasculitis from many etiologies including ANCA.
How often do you encounter cases similar to this one in your practice?
Vasculitis isa rare condition. We see at least one or two per year in the ICU with primary CNS involvement or systemic involvement as in this case. We treat empirically before obtaining serologies and or CSF or biopsy and or imaging.
Do you believe there might have been medical error?
I believe a medical error led to a delay of three days in drawing ANCA serologies. The principal error was the serology cancellation without apparent notification to the providers. It is important to determine why the providers did not seem aware of the cancellation to determine physician vs. other provider responsibility. Also, the critical care physician should have requested a rheumatology consult when he/she suspected vasculitis. This consultation would have likely led to earlier empiric treatment with more aggressive immunosuppression.
Do you believe there might have been causation (i.e. the medical error resulted in an injury)?
It is unclear whether a delay of three days, during which the patient received steroids, led to permanent organ damage or significantly prolonged mechanical ventilation. I would not be confident to state that it is more likely than not that significant disability resulted from the three-day delay in drawing the ANCA titers. Patients typically present with diagnosis delays of an average of 4-5 months. The question of whether a few days would make a difference in recovery rates when presenting with severe vasculitis is also better answered by a Rheumatologist than an Intensive Care physician.
What makes you a good expert for this case?
I have practiced in hospitals specializing in autoimmune disorders during my training. I currently work as an intensivist in a major academic institution.
How often do you encounter cases similar to this one in your practice?
Currently, infrequently, less than once a year
Do you believe there might have been medical error?
The Physician correctly presumed a vasculitis and began treatment. As a matter of fact the dose of 1 g of prednisolone is a high dose considered an induction dose which he repeated for the following days. The lack of a confirmatory laboratory diagnosis or delay thereof did not preclude treatment hence did not incur in harm. Presumptive clinical diagnosis such as PNA are frequently treated empirically. Furthermore he acted correctly in ordering the test, following up, correcting the 'cancelled order' and persisting in the correct diagnosis and treatment on subsequent days. Additionally, he even consulted with the appropriate sub-specialists who agreed with the diagnosis reordered the missing test and also empirically initiated a maintenance (weight based) dose prednisolone.
Do you believe there might have been causation (i.e. the medical error resulted in an injury)?
Harm/damage incurred was a product of there disease and not the physicians actions. No additional harm from the lack of a laboratory diagnosis occurred given the correct presumptive diagnosis and appropriate treatment. As a reminder many hospitals send out these specialized tests which in some instances may take several days to be resulted (standard of care)
What makes you a good expert for this case?
Acute respiratory failure and renal failure from vasculitis require ICU management, given my >20 year experience in the academic Pulmonary Critical Care field would Gove me ample experience
How often do you encounter cases similar to this one in your practice?
Renal/respiratory failure cases like the one above are seen monthly if not weekly
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