Doctors should suspect granulomatosis with polyangiitis (GPA) in cases of isolated, acute sinusitis that does not improve with treatment, especially in young patients, a case report suggests.
The study, “Very painful acute frontal sinusitis revealing granulomatosis with polyangiitis,” was published in the journal European Annals of Otorhinolaryngology, Head and Neck Diseases.
GPA is a type of ANCA-associated vasculitis, characterized by the appearance of granulomas, which are masses of immune cells. Most times, GPA affects organs such as the head, neck, lungs, and kidneys, instead of causing a single symptom.
Researchers in France reported the case of a 27-year-old man who came to the hospital with acute sinusitis, which had persisted in spite of treatment, as the only symptom of GPA.
The man went to the hospital because he had severe headaches associated with sinusitis; he had no previous history of this condition.
Lab exams showed high C-reactive protein (a marker of inflammation) and high neutrophil count, which are the immune cells attacked by ANCAs that cause vasculitis when they burst.
The man received several courses of antibiotics, but the symptoms persisted. A computed tomography (CT) scan showed that the inner lining of the nasal sinuses was inflamed and swollen, and that the left sinus was obstructed by mucus.
The patient underwent surgery to drain his left sinus, which improved the symptoms. Mucus examination showed no bacteria.
The symptoms came back some after the surgery. The doctors performed a second drainage, but the patient continued to have severe headaches and inflammation in the inner lining of the sinuses, especially in the left sinus.
Since sinusitis did not respond to medication or surgery, the doctors suspected that GPA was causing it.
Further exams showed that the patient had ANCAs against proteinase 3 (PR3) and small amounts of blood in the urine, indicating kidney involvement. At that time, the man also developed systemic symptoms like weakness, anorexia leading to 12-kilogram weight loss (about 26 pounds) in four months, night sweats, cough, joint pain, and inflammation of the cartilage of the nose.
Imaging exams showed a mass and a nodule in the lungs. A biopsy of the nodule showed it was a granuloma and confirmed GPA diagnosis.
The man received a combination of corticosteroids and Rituxan (rituximab) that improved the symptoms and lab exams. A CT scan performed a year-and-a-half after diagnosis showed that the patient no longer had sinusitis.
“Sinusitis is a common clinical presentation of GPA, but the clinical features are usually bilateral [affecting both sinuses equally] with a chronic course over several years. Isolated, unilateral acute sinusitis is an exceptional clinical presentation of GPA,” the researchers wrote.
The investigators proposed that “the diagnosis of GPA must be considered in a young patient with no notable history, in the presence of very painful, persistent and recurrent unilateral acute sinusitis despite appropriate treatment and the patient must be examined for the presence of associated systemic signs,” they continued.
“These sudden-onset forms of GPA in young subjects are characterized by their severity and their rapidly deteriorating course. Early diagnosis and urgent treatment are essential determinants of the prognosis,” they concluded.