Study Reveals Difficulties in Diagnosing GPA for Patients in Saudi Arabia

Marisa Wexler, MS avatar

by Marisa Wexler, MS |

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The case of a man in Saudi Arabia with granulomatosis with polyangiitis (GPA), a form of ANCA-associated vasculitis, highlights the difficulties that can occur in diagnosing this disease, a case study reports.

The report is one of the few documented instances of GPA in Saudi Arabia.

The study, “Granulomatosis with Polyangiitis Presenting as Refractory Chronic Cough, Manifesting by Massive Alveolar Haemorrhage — A Rare Case Report,” was published in the International Medical Case Reports Journal.

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A 36-year-old man who had been a smoker for two decades went to the hospital with complaints of a frequent cough and intermittent shortness of breath. He also complained of a sore throat and generalized body aches, which had been present for the prior six months.

Over those six months, the man had received several courses of antibiotics on the assumption that his symptoms were likely the result of an infection. However, the symptoms failed to improve.

Imaging of the man’s lungs revealed marked areas of damage and inflammation, and blood tests showed abnormally high levels of certain inflammatory markers.

Since the man was fairly young and did not have other apparent symptoms, his doctors assumed that an infection was the most probable cause of these symptoms. Tests for certain infections — including tuberculosis and Middle East respiratory syndrome coronavirus (MERS) — came back negative.

Based on these findings, the man was diagnosed with atypical pneumonia — essentially meaning an infection of the lower lungs — and was sent home on a cocktail of antibiotics.

A few days later, the man returned to the hospital reporting that his symptoms had worsened: He had started to cough up blood and had developed a rash on his legs as well as substantial joint pain.

These symptoms prompted his doctors to consider that he might have a systemic vasculitis, a condition characterized by inflammation in the blood vessels, such as ANCA-associated vasculitis.

Additional blood testing revealed that the patient was positive for ANCA, the self-reactive antibodies that drive the disease. Based on his specific symptoms and laboratory findings, the man was ultimately diagnosed with GPA.

He was given several treatments, including rituximab, plasma exchange (a procedure that replaces the liquid portion of blood to eliminate antibodies in circulation), and corticosteroids. His symptoms improved immediately. Six months after leaving the hospital, he underwent repeat imaging, which showed no signs of lung damage.

“Granulomatosis with polyangiitis is a rare systemic vasculitis that should be considered in young patients presenting with refractory chronic cough and upper respiratory tract symptoms,” the researchers concluded.

They added that, while there is plenty of literature on GPA in Europe and the U.S., “there are very few reports of granulomatosis with polyangiitis from Saudi Arabia.”