A group of autoimmune diseases called anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) is the subject of a preprint under consideration at Pediatric Rheumatology.

ANCA-AAV is characterized by damage to small blood vessels causing inflammation, whereas ANCAs are antibodies that trigger cells in the immune system called neutrophils and overly activate them.

ANCA-AAV may cause many varied symptoms since small blood vessels are situated throughout the body.

About the Three Subtypes

Patients can be placed into three major subtypes in accordance with their symptoms:

• Granulomatosis with polyangiitis (GPA) is a rare disorder mostly involving damage to the kidneys and respiratory tract
• Microscopic polyangiitis (MPA) (a rare vasculitis) is a condition causing inflammation in small blood vessels
• Eosinophilic granulomatosis with polyangiitis (EGPA) symptoms most often involve the kidney, respiratory tract, joints, and the skin

ANCA is seldom found in children. Estimates are three to four cases per million people. Cardiac abnormalities are even rarer in this population.

Two Cases of Cardiac Valvular Abnormalities in GPA

Case #1 involved a sixteen-year-old male who had a six-week history of cough, fatigue, bloody nose, loss of weight, fever, and dark urine.

He had hypertension, and his blood pressure ranged from 120 to 166/44-92. Due to his persistent coughing, he underwent a CT scan. The scan showed ground-glass opacity (hazy gray areas) indicating density inside the lungs.

Consultations were then conducted with Infectious Disease, Rheumatology, and Nephrology. His C-ANCA pattern was found to be positive.

He then underwent a comprehensive infectious workup including serum testing, results of which were negative. His COVID-19 swab test was negative. The patient was medicated for hypertension and to improve the function of his heart muscle.

Day 3 in the Hospital

On his third day in the hospital, he underwent a renal biopsy which showed focal necrotizing, an inflammation of blood vessel walls. Focal necrotizing can disrupt normal blood flow resulting in muscle and skin damage. Necrosis (death of cells in an organ) may also occur.

The patient’s renal biopsy also showed pauci-immune crescentic glomerulonephritis, which is common in ANCA-AAV.

During the renal biopsy, doctors found Mobitz II heart block leading to his cardiac evaluation. Second-degree type 2 heart block affects the heart’s electrical conduction system.

When the echocardiogram was completed, the patient experienced an ischemic event resulting in a facial droop together with sensations on the left side. He was diagnosed with a thromboembolic stroke.

An angiogram scan of his head confirmed that he had an area of dead tissue caused by a lowered blood supply.

ANCA-Vasculitis Protocol

His physicians followed ANCA-vasculitis protocol due to AAV and cardiac lesions. AAV was kept on the differential (a list of possible diseases). Cardiac screening is also important for newly diagnosed AAV patients.

Four months after the onset of his disease, he was doing clinically well with rituximab and was asymptomatic.

Continued in Part Two