Two Teenage Boys Diagnosed With ANCA-Associated Vasculitis, Part Two

Continued From Part One

Case #2

The second case involved a sixteen-year-old male in the ICU unit with suspected bacterial hypoxic respiratory failure associated with viral pneumonia.

The teen’s history included painful elbows and knees that had been resolved with NSAID. He was admitted to ICU with congestion, cough, fever, and myalgias. He tested negative for COVID-19.

The medication he was given, azithromycin, did not improve his condition. He tested positive for influenza A and was given doxycycline and amoxicillin but became weak and had difficulty breathing. His heart rate was over 100 beats per minute (tachycardia).

The patient received a repeat chest X-ray which indicated the development of a collapsed lung (bilateral pneumothoraces). Dense opacities were found in both of his lungs.

On the second day of his hospitalization, the patient was given cardiopulmonary support for respiratory failure. Test results showed pulmonary hemorrhage which occurs with blood leaking into the lung from vessels in airways or windpipes.

After additional tests, the patient was placed on VV ECMO, an essential tool for patients who have serious pulmonary and cardiac dysfunction. An echocardiogram was conducted, and cardiology was consulted.

The patient was treated with a broad spectrum of antibiotics (Vancomycin, Cefepime, Gentamicin) to combat pneumonia or infections to the heart or heart valves (endocarditis).

The ICU team placed the patient on methylprednisolone from day 2 to day 4 with positive results.

On days six and seven, upon examination, the patient developed hemorrhages and small red spots (petechiae) consistent with inflammation of minor blood vessels (leukocytoclastic vasculitis).

On day 9 in the hospital, the team consulted rheumatology with evidence of additional blood in the urine (hematuria). The infectious workup results were negative. There was a concern for a rheumatologic disorder.

The patient’s ANCA panel was sent for evaluation that day. Results were returned within five days. They were consistent with the pattern for c-ANCA.

By day 11, the patient’s renal condition had deteriorated. He presented a higher than normal level of potassium in his bloodstream (hyperkalemia) requiring Veno-Venous hemofiltration. This is a temporary treatment for patients unable to tolerate hemodialysis. At the same time, the patient began treatment with methylprednisolone 500mg and five days of plasmapheresis.

About Plasmapheresis

The five-day course of plasmapheresis consisted of withdrawing blood plasma and separating it into cells and plasma. The cells are returned to the bloodstream by way of retransfusion.

Plasmapheresis is especially suited to the removal of antibodies to treat autoimmune conditions.

The patient’s kidney and lung function improved once he began treatment with plasmapheresis and pulse methylprednisolone.

The temporary Hemofiltration CVVH was discontinued on day 19. On his 20th day in the hospital, the tracheostomy tube was removed (decannulated).

Day 22 began with a renal biopsy supporting a diagnosis of granulomatosis with polyangiitis (GPA), an uncommon disorder causing inflammation of blood vessels. It is one of the vasculitis disorders that slows blood flow to various organs.

The patient is now eighteen months post-diagnosis and on maintenance doses of 100mg of rituximab every 6 months.

The original report is available here.


The two patients eventually experienced an improvement in their conditions while being treated with high-dose methylprednisolone, rituximab, and plasma.

Only five pediatric cases of cardiac valvular involvement have been associated with GPA.

With respect to ANCA, if cardiac involvement is discovered, it is imperative to conduct an infectious workup. The purpose is to identify infectious diseases and confirm ANCA-associated vasculitis before initiating immunosuppressive therapy.

Rose Duesterwald

Rose Duesterwald

Rose became acquainted with Patient Worthy after her husband was diagnosed with Acute Myeloid Leukemia (AML) six years ago. During this period of partial remission, Rose researched investigational drugs to be prepared in the event of a relapse. Her husband died February 12, 2021 with a rare and unexplained occurrence of liver cancer possibly unrelated to AML.

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