1st Case to Report Child With AAV, CNS Symptoms After COVID-19
Virus may trigger autoimmune symptoms in susceptible patients, study suggests
A 13-year-old girl developed ANCA-associated vasculitis (AAV) with major involvement of the central nervous system after COVID-19, a case study reports.
“To our knowledge, this is the first reported pediatric case of ANCA-associated vasculitis with predominant central nervous system involvement after COVID-19 infection,” the researchers wrote.
The girl had a history of another autoimmune disease called immune thrombocytopenic purpura, which may have increased her risk of autoimmune-related diseases, the team noted. Also, immunosuppressive therapy effectively lessened her AAV symptoms.
The case report, “c-ANCA-associated vasculitis with predominant CNS demyelination after COVID-19,” was published in Archives de Pédiatrie.
In about 15% of AAV patients, the central nervous system is affected
AAV is a group of autoimmune diseases caused by the production of anti-neutrophil cytoplasmic antibodies, or ANCAs, that mistakenly target the body’s neutrophils, a type of white blood cell. This results in inflammation and damage to small blood vessels, including those in the lungs, kidneys, and skin.
In about 15% of AAV patients, the central nervous system (CNS; brain and spinal cord) is also involved and may include symptoms such as headache, blood clot-induced stroke, brain bleeding, conditions affecting the brain’s structure or function, and, rarely, spinal cord-related problems.
Diagnosing AAV can be challenging, and “CNS symptoms in AAV may hinder early diagnosis, causing treatment delays and disease progression, which lead to relapse or even death,” the researchers wrote.
SARS-CoV-2, the virus that causes COVID-19, “has been shown to trigger the presentation or exacerbation of autoimmune diseases in genetically susceptible patients,” the researchers wrote.
Six cases of AAV after COVID-19 infection have been reported, all involving adults and mostly affecting the kidneys and lungs. No such cases have shown CNS manifestations.
Now, a team of researchers at the Fattouma Bourguiba University Hospital, in Tunisia, described what they believe to be the first pediatric case of AAV with predominant CNS involvement after SARS-CoV-2 infection.
A 13-year-old girl was admitted to their pediatric department in June 2021 due to walking difficulties and numbness in her legs that had begun three weeks before. She had a history of chronic immune thrombocytopenic purpura, an autoimmune disease caused by abnormal immune attacks against platelets, the cell fragments that help blood clot.
The girl had been exposed to SARS-CoV-2 through her parents four weeks before, and all had experienced mild COVID-19 symptoms.
[SARS-CoV-2, the virus that causes COVID-19] has been shown to trigger the presentation or exacerbation of autoimmune diseases in genetically susceptible patients
Physical examination found signs of CNS disorder in girl
Physical examination showed slightly reduced muscle strength, sensation, and reflexes in her right foot, suggesting a CNS disorder. Blood counts were normal, as well as kidney and liver function.
Magnetic resonance imaging (MRI) showed a thickening of the spinal cord and lesions in the neck region, as well as demyelinating lesions in the brain. Demyelination refers to the damage and/or loss of myelin, the protective sheath that covers nerve fibers and is key for fast signal transmission.
The girl also had abnormally high levels of immune cells and proteins in the cerebrospinal fluid, the liquid that surrounds the brain and spinal cord.
A lung MRI scan revealed infiltration, meaning that a substance denser than the air, such as blood or protein, was present in the lungs. She was negative for SARS-CoV-2 and bacterial infections.
Based on these results, the girl’s initial diagnosis was CNS demyelination after COVID-19 infection. She received immunosuppressive treatment, including five days of high doses of steroids, administered directly into the bloodstream, followed by oral prednisolone.
However, the girl experienced seizures two weeks later, requiring admission to the intensive care unit.
Additional MRI scans revealed thickening of the dura matter (the thick membrane surrounding the brain and spinal cord), spinal cord enlargement and inflammatory lesions in the neck and chest regions, and demyelinating lesions in the brain.
A whole-body computed tomography (CT) scan showed infiltrations in both lungs, mild enlargement of the liver and spleen, and enlargement of several lymph nodes (immunological structures) in the abdomen region.
Furthermore, a blood test showed high levels of ANCAs, but not of abnormal antibodies known to be associated with other autoimmune conditions.
A few days later, the girl developed palpable purpura (elevated, firm, bleeding bumps in the skin) and a type of skin rash.
History of blood autoimmune disorder may have increased girl’s risk for AAV
She was treated with high doses of into-the-vein methylprednisolone (1 g/day) for five days, followed by oral prednisolone and rituximab (sold as Rituxan in the U.S., MabThera in Europe, with biosimilars available). She underwent physical therapy and rehabilitation, resulting in a gradual reduction of neurological symptoms.
New MRI and CT exams showed regression of the demyelination lesions and reduction of lung infiltrations. The ANCA test was also negative after two months of therapy.
Overall, the girl met criteria suggestive of an AAV diagnosis, including lung and skin symptoms, elevated ANCA levels, and symptom reduction with rituximab.
“Notably, the history of chronic immune thrombocytopenic purpura in this patient may increase the risk of other autoimmune diseases and suggests a possible genetic predisposition,” the researchers wrote.
This study describes for the first time a pediatric case of AAV after COVID-19 with CNS symptoms.
“The diagnosis of new-onset AAV with predominant CNS involvement can be challenging in COVID-19 patients because of the similarity in symptoms and clinical manifestations of both diseases,” the team wrote, adding that “timely diagnosis and treatment are crucial for this life-threatening disease.”
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