Lung Lesion Removal Leads to GPA Remission in Woman, 77, in Japan

Immunosuppressants were not needed after lung lesion surgery in case report

Patricia Inacio, PhD avatar

by Patricia Inacio, PhD |

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Surgical removal of an inflammatory lung lesion led to remission of granulomatosis with polyangiitis (GPA) — a type of ANCA-associated vasculitis — in a 77-year-old woman in Japan, without the use of immunosuppressive therapy, a case report describes.

In cases of GPA without severe lesions, a “reevaluation of the disease activity after diagnostic resection is crucial for considering the need for immunosuppressive therapy,” according to the report authors.

The report, “Remission of Granulomatosis with Polyangiitis Only After Resection of a Pulmonary Nodule,” was 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 cause damage to 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 both medium-sized and small blood vessels. Most commonly, the disease affects the lungs, the upper respiratory tract (nose, trachea, and ears), and kidneys. In GPA, inflammation sites generally 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 work as an extracellular web that traps and kills disease-causing microbes, but can also release a series of molecules that contribute to autoimmunity and tissue damage in AAV.

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

GPA patient in remission after surgery without the use of immunosuppressants

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

The patient, a 77-year-old woman, had cough, nasal discharge, sputum (phlegm), and an overall poor health condition in the five months prior to her hospitalization. She developed fever and left ear fullness.

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

Upon hospital admission, she was feverish and hearing impaired in her left ear. Levels of C-reactive protein were still elevated and blood tests showed she was positive for myeloperoxidase (MPO) antibodies, one of the main types of self-reactive antibodies seen in people with AAV. Tests for infection and tumor markers came back negative.

Her lung lesions were surgically removed and a follow-up analysis revealed the presence of classical GPA features, including the presence of cells in blood vessel walls and granulomatous lesions. In addition, neutrophils and NETs were also detected.

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

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

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One year after surgery, the woman showed no evidence of relapse and her MPO-ANCA levels were still decreasing.

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

“We hope that this report provides valuable suggestions concerning the pathogenesis [causes] of AAV,” they wrote. “In cases with no other severe lesions, it may be best to reevaluate the disease activity and the need for immunosuppressive therapy after diagnostic resection of the lesion.”