Rare SLE-AAV Overlap Syndrome May Cause Stroke, Case Report Shows

While rare, ANCA-associated vasculitis (AAV) may overlap with systemic lupus erythematosus (SLE) — the most common form of lupus — and manifest as an hemorrhagic stroke caused by a weakened blood vessel in the brain, a case study shows.

The study, “Systemic lupus erythematosus and antineutrophilic cytoplasmic antibody-associated vasculitis overlap syndrome complicated by subarachnoid hemorrhage: case-based review,” was published in the journal Rheumatology International.

SLE is an autoimmune disease where the immune system mounts attacks against a person’s own cells. The condition can involve the skin, kidneys, lungs, nervous system, and blood system, among others.

AAV is also an autoimmune condition, but it is characterized by damage to small- and medium-sized blood vessels.

Both diseases are rare, but in some cases, patients may present features of both. This is called SLE-AAV overlap syndrome and mostly presents as evolving kidney inflammation.

However, in rare cases, patients have also experienced neurological symptoms, particularly cerebral ischemia (when not enough blood reaches the brain). This can cause an ischemic stroke and severe brain damage.

Now, researchers reported the case of a 40-year-old who had a hemorrhagic stroke — when blood spills into or around the brain and creates swelling and pressure, damaging cells and tissue — as a manifestation of SLE–AAV overlap syndrome.

The patient, an African-American women, went to a hospital after waking up with a severe right-sided headache and weakness. She had a history of mild developmental delay and hypertension.

After a first evaluation, physicians found she had had a hemorrhagic stroke caused by an aneurysm — a bulging blood vessel that ruptured. Because of the severity of her condition, she was referred to a specialized clinic for immediate intervention.

A computed tomography (CT) scan showed a weak spot in an artery in the right side of the brain. Clinicians were able to stop the bleeding; the woman was discharged to an inpatient rehab facility.

Two weeks later, however, the woman returned to the hospital with abdominal pain, non-bloody vomiting, bloody cough, low levels of platelets in the blood, and high creatinine levels, suggestive of acute kidney injury.

Researchers found red blood cells in her urine and abnormal tissue structures in her kidney consistent with intrinsic renal disease. Chest CT scans showed several nodular alterations and accumulation of liquid in the left lung cavity.

After removing 700 ml of liquid from her lungs, the woman’s cough improved significantly. However, the team found high levels of immune cells in this liquid without any signs of inflammation.

At this point, blood tests came back positive for anti-myeloperoxidase (MPO), ANCA, and anti-nuclear antibodies — a sign of SLE. Also, a kidney biopsy revealed immune-mediated severe kidney inflammation and scarring (known as glomerulonephritis).

The patient was first diagnosed with AAV and treated with methylprednisolone and hemodialysis because of impaired renal function. She then received remission induction treatment for AAV. While it was well-tolerated and alleviated some of her symptoms, kidney function was still compromised and she developed a rash on her trunk and extremities.

Physicians suspected SLE, which was confirmed with a skin biopsy.

She continued on hemodialysis and the remission induction treatment. For the next 15 months, her condition remained stable without any new brain blood vessel lesions or hemorrhages.

“The SLE-AAV overlap syndrome is an emerging diagnosis with only few documented cases in the literature and without a strictly defined [diagnosis] criteria,” researchers said. However, the reported neurological presentation is not well-known.

“Clinicians who encounter patients with neurologic signs that present with symptoms and a [blood biochemical] profile that correspond to both SLE and AAV criteria should consider the association between SLE-AAV overlap syndrome and a hemorrhagic stroke, specifically subarachnoid hemorrhage,” they stated.