J Rheum Dis.  2020 Jul;27(3):203-208. 10.4078/jrd.2020.27.3.203.

Antineutrophil Cytoplasmic Antibodies Negative Microscopic Polyangiitis With Initial Pulmonary Manifestation

• Lee JS
• Oh MS
• Chung JH
• Lee S
• Kwon JW

• KMID: 2504035
• DOI: http://doi.org/10.4078/jrd.2020.27.3.203

Abstract

Microscopic polyangiitis (MPA) is a small vessel vasculitides mostly associated with anti-neutrophil cytoplasmic antibodies (ANCA). The kidney is the most commonly affected organ in MPA. We report the case of a 9-year-old girl with ANCA-negative MPA who initially presented with respiratory symptoms, including cough, sputum, and dyspnea. Based on her symptoms, atypical pneumonia was suspected. Also, childhood interstitial lung disease was considered based on findings seen on chest CT. Despite initial improvement of symptoms with oral corticosteroid therapy, dyspnea with initiation of corticosteroid tapering was noted. A final diagnosis of MPA was made after lung biopsy. ANCA was negative in both the initial and repeat blood tests. Oral cyclophosphamide and prednisolone treatments led to full remission. Since then, the patient has been treated with low dose prednisolone and azathioprine for maintenance. A good treatment response was achieved and her clinical symptoms, pulmonary functions, and radiologic findings have since improved. Thus, early and precise diagnosis of MPA is crucial for remission induction and prevention of symptom relapse.

Keyword

Microscopic polyangiitis; Interstitial lung diseases; Antineutrophil cytoplasmic antibodies; Child

Figure

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  Figure 1 Chest X-ray & chest computed tomography (CT) before lung biopsy. (A) Initial chest X-ray and CT scan: Initial chest X-ray (09/11/2016) showed peribronchial hazy infiltrations in both lower lung zones. Chest CT scan showed diffuse bronchocentric ground glass opacities and consolidation in bilateral lungs suspected to atypical pneumonia. (B) Follow-up chest X-ray and CT scan in the period of symptom worsening: Chest X-ray showed an interval improved aeration but residual streaky and granular infiltrations. Chest CT scan showed a resolution of previously noted ground glass opacities in both lungs, leaving diffuse fibro-streaky changes and some consolidations.

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  Figure 2 Pathologic findings of the biopsy specimen. (A) Surgical lung biopsy specimen shows subpleural and intraalveolar hemorrhage (H&E, ×2). (B) At higher magnification, fibrin deposition, hemosiderin-laden macrophages in alveolar spaces and capillaritis associated with neutrophils can be seen (H&E, ×100).

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  Figure 3 Changes in pulmonary function tests according to treatment. Gradual improvement of forced vital capacity and diffusion capacity is noted after remission induction treatment. DLco: carbon monoxide diffusing capacity, FVC: forced vital capacity, VATS: video-assisted thoracoscopic surgery, Pd: prednisolone, bid: twice daily, qd: once daily.

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  Figure 4 Chest X-ray & computed tomography (CT) scan performed at the one year after treatment. (A) Chest X-ray showed no active lung lesion except for postoperative changes in right lung. (B) Chest CT scan showed disappeared previously-noted diffuse fibrostreaky changes and consolidation in both lungs.

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