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Case Reports
. 2024 May 1;63(9):1253-1260.
doi: 10.2169/internalmedicine.2512-23. Epub 2023 Sep 15.

Slowly Progressive ANCA-associated Glomerulonephritis with Strong Mesangial MPO Deposits Following a Diagnosis of Interstitial Lung Disease

Hitoshi Anzai  1 , Yuki Suzuki  2 , Masaki Ueno  1 , Shinichiro Asakawa  1 , Michito Nagura  1 , Shigeyuki Arai  1 , Osamu Yamazaki  1 , Yoshifuru Tamura  1 , Ryuji Ohashi  3 , Shigeru Shibata  1 , Yoshihide Fujigaki  1
Affiliations
Case Reports

Slowly Progressive ANCA-associated Glomerulonephritis with Strong Mesangial MPO Deposits Following a Diagnosis of Interstitial Lung Disease

Hitoshi Anzai et al. Intern Med. .

Abstract

An elderly woman showed positive conversion of myeloperoxidase (MPO)-antineutrophil cytoplasmic antibodies (ANCAs) following the diagnosis of interstitial lung disease (ILD) and glomerular hematuria and subsequently experienced slowly progressive glomerulonephritis. A kidney biopsy revealed chronic damage and necrotizing crescentic glomerulonephritis with mesangial MPO deposits. After corticosteroid treatment, the patient's urinalysis results and MPO-ANCA titers almost normalized and her renal function stabilized. This case is similar to recently reported cases of slowly progressive ANCA-associated glomerulonephritis. ILD likely triggered the production of MPO-ANCAs, and the accumulation of MPO deposits in the glomeruli may have contributed to the progression of her renal disease.

Keywords: ANA; ANCA-associated glomerulonephritis; idiopathic interstitial pneumonia; myeloperoxidase.

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Conflict of interest statement

The authors state that they have no Conflict of Interest (COI).

Figures

Figure 1.
Figure 1.
Plain CT images of the chest in the axial (A and B) and coronal (C) planes. A: Right lung dominant reticulation in the bilateral lower lungs and multiple ground-glass opacities in the right lung are found. B and C: Slightly worsening reticulation in the lower right lung (B, C) with the development of contraction (C) in comparison to the previous findings.
Figure 2.
Figure 2.
The clinical course after the first hospitalization. u-OB: urinary occult blood on a urine dipstick, u-prot: urinary protein on urine dipstick, sCr: serum creatinine, CRP: C-reactive protein, MPO-ANCA: myeloperoxidase-antineutrophil cytoplasmic antibody
Figure 3.
Figure 3.
Light micrographs of renal tissues. A: A globally sclerotic glomerulus due to fibrotic crescent formation is observed. Original magnifcation ×400. Periodic acid-Schiff staining. B: A glomerulus exhibits circumferential cellular crescent. The interlobular artery shows no vasculitis, but there is a mild degree of atherosclerotic changes. Original magnification ×400. Elastica-Masson staining. C: Two glomeruli show segmental necrosis with the exudation of fibrin-like material (asterisks) and inflammatory cell infiltration both within and outside the glomerular capillary walls. A mild degree of tubulitis is found (arrow). Original magnifcation ×200. Periodic acid-Schiff. D and E: The enlarged photos of the upper left glomerulus in Fig. 3C (D) and the right glomerulus in Fig. 3C (E). Segmental necrosis of glomerular capillary walls with the exudation of fibrin-like material into Bowman’s space (asterisks). Original magnifcation ×400. Periodic acid-Schiff.
Figure 4.
Figure 4.
Immunofluorescence study. Immunofluorescence staining shows weak positivity for IgG (B), IgM (C), C3 (E), κ light chain (F) and λ light chain (G) and less weak positivity for IgA (A), mainly in the capillary areas, but negativity for C1q (D). Positive nuclear IgG (H), κ light chain (F) and λ light chain (G) staining is seen. Staining with anti-human myeloperoxidase (MPO) antibodies shows strong positivity for MPO, mainly in the mesangial areas (I). In the control kidney biopsy sections from a patient with minimal change nephrotic syndrome, MPO is not detected in the glomerulus (J). In the kidney biopsy sections from a patient with immunoglobulin A nephropathy, positive MPO staining is observed in a small round pattern in the glomerulus, likely indicating the presence of neutrophils known to express MPO (K). Original magnification ×200. The primary antibodies were polyclonal rabbit anti-human MPO antibody (Nr A 0398 Dako Denmark, Glostrup, Denmark), and the secondary antibody, Alexa Fluor TM 488 conjugated polyclonal donkey anti-rabbit IgG heavy and light chain antibody (Thermo Fisher Scientific, Tokyo, Japan).
Figure 5.
Figure 5.
Electron micrograph of a glomerulus. A: There is no mesangial cell proliferation and no thinning of glomerular basement membrane. A, B: Electron-dense deposits (EDDs) (asterisks) are found in the mesangial areas but no EDDs are seen in the subepithelial areas. Partial foot-process effacement and mild endothelial cell swelling are seen. A: Bar=10.0 μm. B: Bar=2.0 μm.
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