Surgical removal of an inflammatory lung lesion resulted in remission of granulomatosis with polyangiitis (GPA) – a type of ANCA-associated vasculitis – in a 77-year-old woman in Japan, without the use of treatment immunosuppressant, describes a case report.

In the case of GPA without severe lesions, a “reassessment of disease activity after diagnostic resection is crucial to consider the need for immunosuppressive treatment”, according to the authors of the report.

The report, “Remission of granulomatosis with polyangiitis only after resection of a pulmonary nodulewas published in the journal Internal Medicine.

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Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of inflammatory conditions that damage small and medium-sized blood vessels. ANCAs, self-reactive antibodies that bind to immune cells called neutrophils, are the drivers of this damaging inflammation.

GPA is a type of AAV marked by inflammation of the walls of medium and small blood vessels. Most often, the disease affects the lungs, the upper respiratory tract (nose, trachea and ears) and the kidneys. In GPA, the sites of inflammation usually contain masses of immune cells called granulomas.

The causes of GPA are poorly understood, but studies suggest that activated neutrophils and neutrophil extracellular traps (NETs) play a role in AAV. NETs are structures formed by neutrophils that normally function as an extracellular web that traps and kills pathogenic microbes, but can also release a series of molecules that contribute to autoimmunity and tissue damage in AAVs.

First-line treatment for GPA includes glucocorticoids, immunosuppressants, and antibody therapies. Patients often continue with immunosuppressive therapy to prevent the disease from coming back (relapse).

GPA patient in remission after surgery without use of immunosuppressants

In the report, researchers in Japan described the case of a GPA patient who achieved remission with resection (surgical removal) of a lung lesion and without the use of immunosuppressive therapy.

The patient, a 77-year-old woman, had cough, runny nose, sputum (phlegm), and poor general health in the five months preceding his hospitalization. She developed fever and fullness in her left ear.

Blood tests revealed elevated levels of C-reactive protein, a marker of generalized inflammation. A CT scan showed two shadows in his right lung. Treatment with the antibiotic azithromycin failed to alleviate her symptoms.

When she was admitted to the hospital, she was feverish and hard of hearing in her left ear. C-reactive protein levels were still elevated and blood tests showed it was positive for anti-myeloperoxidase (MPO) antibodies, one of the main types of auto-reactive antibodies seen in people with AD. AAV. Infection tests and tumor markers came back negative.

His lung lesions were surgically removed and a follow-up scan revealed the presence of classic GPA features, including the presence of cells in the walls of blood vessels and granulomatous lesions. Additionally, neutrophils and NETs were also detected.

These results were compatible with a GPA diagnosis. Her treatment plan included high-dose glucocorticoids and immunosuppressants. However, the patient’s symptoms (cough, fever, and ear fullness) and inflammation were markedly reduced after resection of the lung lesions before any additional treatment was given.

Eventually, her symptoms resolved completely, with reduced levels of C-reactive protein and MPO-ANCA. Disease activity also decreased, as shown by a decrease in the Birmingham Vasculitis Activity Score from seven to zero.

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A year after surgery, the woman showed no signs of relapse and her MPO-ANCA levels continued to decline.

“This report does not recommend surgery as a treatment for AAV,” its authors wrote. Rather, they suggest that GPA may be fueled by a vicious cycle of NETs and ANCAs and that “reduction of activated neutrophils and NETs may induce AAV remission by arresting this cycle.”

“We hope that this report will provide valuable suggestions regarding the pathogenesis [causes] of the AAV,” they wrote. “In cases without other serious lesions, it may be best to reassess disease activity and the need for immunosuppressive therapy after diagnostic resection of the lesion.”