ANCA Vasculitis Can Develop in Scleroderma Patients in Rare Cases, Study Reports

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by Alice Melão |

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While rare, patients with scleroderma — a disease that causes skin hardening — can also develop ANCA-associated vasculitis, a case report shows.

The study, “Perinuclear Antineutrophil Cytoplasmic Antibody Positive Glomerulonephritis in a Case of Limited Cutaneous Scleroderma,” was published in the Indian Journal of Nephrology.

Scleroderma, also known as systemic sclerosis or SSc, is chronic autoimmune disease characterized by excess collagen, a main structural component of body tissues including scar tissue. This excess turns tissues thicker and less malleable, changing they ability to function properly.

This disease mostly causes skin scarring, but it can affect other organs. When the kidneys are involved, leading to scleroderma renal crisis, patients can rapidly progress to kidney failure.

In some patients, however, kidney problems may arise from an overlap of scleroderma and other autoimmune conditions, including lupus nephritis and ANCA-associated glomerulonephritis.

Researchers at Kokilaben Dhirubhai Ambani Hospital in Mumbai, India, detailed the case of a scleroderma patient with concomitant ANCA-associated vasculitis (AAV).

The patient, a 58-year-old man, had been diagnosed with limited cutaneous scleroderma five years before coming to their attention. He had a clinical history of Raynaud’s phenomenon — reduced blood flow in the fingers and toes — for the past 20 years and of unusual skin wounds (digital ulcers) for seven years.

He had no signs of pulmonary manifestations, like pulmonary hypertension, often seen in scleroderma patients.

On routine evaluation, the patient showed signs of inflammation and kidney disease, with excess proteins and blood in his urine. He was also positive for ANCA antibodies in his blood.

A kidney biopsy revealed collagen deposits with localized scarring and signs of mild inflammation, but the patient had no deposits of ANCA antibodies in his blood vessels.

He was given three doses of methylprednisolone followed by cyclophosphamide. During follow-up, he used mycophenolate mofetil and oral prednisolone as maintenance therapy, which helped keep inflammation at low levels.

“Our case reinforces the occurrence of AAV in patients with SSc,” the researchers said. “Prompt recognition of vasculitis could lead to the early introduction of an appropriate immunosuppressive regimen resulting in better renal and global survival.”

Researchers suggested that “ANCA testing and kidney biopsy should be performed in patients of SSc with renal involvement” to ensure the best care.