Myeloperoxidase anti-neutrophil cytoplasmic antibody-associated vasculitis with a unique imaging presentation of organizing pneumonia: A case report

Abstract

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) constitutes a group of blood vessel inflammation diseases of autoimmune origin. Myeloperoxidase (MPO) ANCA is closely related to ANCA associated AAV. The MPO-ANCA positive AAV patients have lung involvement at high rates; however, there are only a few reported cases with organizing pneumonia (OP). A 78-year-old man was presented to our hospital due to a fever of 38 °C despite a whole month of antibiotics treatment. Chest computed tomography image revealed restricted consolidations visible in the middle lobe of the right lung and the upper lobe of the left lung, which suggested an OP pattern. MPO-ANCA and urine occult blood tests were positive. Histopathological examination of the transbronchial biopsy revealed OP and mucus plug. Histological findings on renal biopsy showed necrotizing glomerulonephritis related to AAV. The patient was diagnosed with MPO-ANCA positive AAV and was treated with systemic corticosteroid therapy, from which he recovered rapidly. Thus, when diagnosing OP, the possibility of AAV should be considered by ordering patients’ serum ANCA and occult hematuria tests.

Pulmonary involvement of ANCA-associated vasculitis in adult Chinese patients

“…NSIP is found in only 19.1% of ILD patients, which is much lower than a European study [ 7 ]. Meanwhile, 3 cases presented as OP, 2 of them with MPO-ANCA positive and 1 with PR3-ANCA positive, which is a rare and unique imaging manifestation of AAV, with only a few previous cases reported [ 34 – 36 ]. Hence, our study suggests that the imaging findings of AAV can be diverse and OP can be a rare manifestation of AAV-related ILD.…”

Pulmonary Involvement of ANCA-associated Vasculitis in Adult Chinese Patients.

“…NSIP is found in only 19.1% of ILD patients, which is much lower than a European study [6]. Meanwhile, 3 cases presented as OP, 2 of them with MPO-ANCA positive and 1 with PR3-ANCA positive, which is a rare and unique imaging manifestation of AAV, with only a few previous cases reported [36][37][38]. Hence, our study suggests that the imaging ndings of AAV can be diverse and OP can be a rare manifestation of AAV related ILD.…”

Impact of interstitial lung disease on mortality in ANCA-associated vasculitis: A systematic literature review and meta-analysis

“… 11 , 25 A spontaneous improvement case of OP associated MPO-ANCA has been reported, another case also revealed that the deterioration of the general condition improved rapidly via steroid and immunosuppressive treatment. 28 , 29 These data suggest that the prognoses of non-UIP patients are relatively better, which may attribute to their better response to glucocorticoids and immunosuppressants. Therefore, differences of ILD patterns according to AAV subtypes may explain their distinctions in outcome: MPA is associated with poorer prognosis, and GPA is more effective to immunosuppressive therapy while more prone to relapse.…”

“…A few cases were reported that organizing pneumonia (OP) was clinically diagnosed in patients with active AAV. [28][29][30] Combined pulmonary fibrosis with emphysema (CPFE) has also been reported in a few series of MPA. 11,26 Previous studies suggested that ILD seems to be a major prognostic factor in AAV patients.…”