PMC8521163_01
Radiological features during follow-up. (A-C) CT images showing a NSIP + OP pattern and reticular and ground-glass opacities along the bronchovascular bundles with patchy air space consolidation before prednisone treatment. (A) The first chest CT scan at the age of 3 months. (B) The subsequent CT scan at the age of 2 years. (C) The chest CT scan on admission (3 years of age). (D) CT images showing improvement after prednisone treatment for 3 months. (E) CT image showing progressive changes upon relapse of SLE after prednisone treatment for 1 year. (F) CT image showing no significant improvement after treated with prednisone plus CsA. (G-I) CT images showing improvement of NSIP after treatment with prednisone combined with CsA and pirfenidone for 7 months, 13 months and 26 months, respectively.
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High-resolution computed tomography (HRCT) scanning showed reticular and ground-glass opacities along the bronchovascular bundles with patchy air space consolidation following the nonspecific interstitial pneumonia (NSIP) with organizing pneumonia (OP) pattern ( Figure 1C ). On review of the patient's previous medical records, it was found that the patient had been prone to pneumonia since the age of 3 months, and the initial and subsequent chest CT images all showed reticular and ground-glass opacities ( Figures 1A, B ). With radiological and immunological resolution indicating remission ( Figures 1D  and  2 ), the steroid dosage was tapered gradually during outpatient follow-up. Chest HRCT showed progressive changes ( Figure 1E ). Repeat lung scans showed no significant improvement ( Figure 1F ). Furthermore, radiologic findings and pulmonary function improved ( Figures 1G-I   and  Table 1 ).
Clinical course of the patient. CTX, cyclophosphamide; CsA, cyclosporin A; 24h-Upro, 24-hour urinary protein quantity.
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Based on the above findings, a diagnosis of SLE (SLEDAI score 7) with ILD was established, and the patient was started on a prednisone 2 mg/kg/day ( Figure 2 ). With radiological and immunological resolution indicating remission ( Figures 1D  and  2 ), the steroid dosage was tapered gradually during outpatient follow-up.
Histological findings from renal biopsy specimen. Renal biopsy showed one segmental glomerulosclerosis on 50 glomeruli with "full house" pattern depositions within capillary loops.
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A renal biopsy was performed, and histological examination revealed typical lupus nephritis (class V) ( Figure 3 ).