A case of lupus vasculopathy presenting favorable renal outcome

Abstract

Noninflammatory necrotizing vasculopathy, also referred to as lupus vasculopathy, is not infrequently observed in the pathology of lupus nephritis. It affects vessels causing them to become severely narrowed and occluded by a mechanism involving immune complexes. We experienced a 51-year-old woman with lupus nephritis class IV + V, which was accompanied by lupus vasculopathy. Renal biopsy and light microscopy showed eosinophilic hyaline-like material in the afferent and/or efferent arterioles, which narrowed the lumen, and which were positive for IgG by immunofluorescent analysis. Electron microscopy indicated that amorphous material and endothelial detachment occluded the arterioles. These findings were consistent with those of lupus vasculopathy. We treated the patient with steroids and cyclophosphamide. By the day of discharge, her levels of creatinine and proteinuria had undergone partial remission. Although lupus vasculopathy was implied as a lesion with unfavorable renal prognosis, some recent reports suggest its true renal prognosis is not unfavorable necessarily. Nevertheless, lupus vasculopathy is an important finding in diagnosis in contradiction to other vascular legions in systemic lupus erythematosus. In addition, a standard therapy has also not been established. Therefore, it is important to accumulate cases of lupus vasculopathy to determine its prognosis and develop standard treatments.

Keywords: Lupus nephritis; Lupus vasculopathy; Noninflammatory necrotizing vasculopathy.

Fig. 1

Histopathology of the kidney biopsy. a Light microscopy (PAS stain, 200×). Mesangial and endocapillary proliferation, duplication of glomerular basement membrane and/or “wire loop” lesions. b Light microscopy (200×). Cellular crescent and wire loop lesions. c Immunofluorescent study (200×) using frozen specimen. Coarse granular and ring-shaped (band-like) staining along GBM and mesangial deposits of IgG was noted. d–f Electron microscopy (d 6000×, e 12000×, f 30000×). Subendothelial (white arrow) and subepithelial (black arrow) electron-dense deposits are frequently seen. Some deposits revealed parallel arrays (finger print). These findings were consistent with those of lupus nephritis class IV + V

Fig. 2

Histopathology of the kidney biopsy. a Light microscopy (HE stain, 200×). Intraluminal deposits of eosinophilic hyaline-like material without severe inflammatory cell infiltrate was noted at the vascular pole. b Immunofluorescent study using serial paraffin-embedded section (200×). Strong staining of IgG was detected in the material (at the same lesion of the intraluminal deposits. c, d Light microscopy (c PAS stain, d Masson’s trichrome stain, 10×). Hyaline-like material was frequently noticed in the afferent arteriole at the vascular pole (white arrow) and in the consecutive interlobular artery (black arrow). These intraluminal materials were both PAS and fuchsin positive

Fig. 3

Clinical course during hospitalization. Renal function, the level of urine proteinurea, and therapeutic interventions are demonstrated. IV CYC intravenous cyclophosphamide pulse, MP Pulse methylprednisolone pulse, UP/UCr urinary protein per urinary creatinine. The dosage of MP pulse is 1 g/day for 3 days and we used 500 mg/body for IV CYC each time