case_images: PMC9807900_01
case_images: Computed tomography (CT) scanning of the chest and abdomen. (A) Lung CT scan showing scattered ground-glass opacity in both lungs and consolidation in the left lung. (B) Lung CT scan showing enlargement of multiple lymph nodes on the axillae and mediastinum (white arrows). (C) Abdominal CT scan showing enlargement of multiple lymph nodes (white arrows) and enlarged spleen and kidney.
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case_images: PMC9807900_01_fped-10-1077324-g001.jpg
case_images: F1
case_images: Computed tomography (CT) scanning showed scattered ground-glass opacity in both lungs and consolidation in the left lung (Figure 1A), multiple lymphadenopathies on the axillae, mediastinum, groins, and the root part of the mesentery, and enlarged kidneys and spleen (Figures 1B,C).
case_images: Immunoglobulin levels of the patient. (A) Initial serum immunoglobulin G (IgG) level was significantly elevated and remained at the upper limit of normal after treatment. (B) Serum immunoglobulin A (IgA) levels were persistently higher than normal. (C) Serum immunoglobulin M (IgM) levels were persistently higher than normal. The dotted line was the normal reference value.
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case_images: PMC9807900_01_fped-10-1077324-g002.jpg
case_images: F2
case_images: The serum immunoglobulin levels were all higher than normal: IgG 19.96 g/L (5.04-14.65) (Figure 2A), immunoglobulin A (IgA) 2.33 g/L (0.27-1.95) (Figure 2B), and IgM 4.5 g/L (0.24-2.1) (Figure 2C).
case_images: PMC9807900_02
case_images: Results of ultrasound and LSGB. (A,B) Ultrasound scan showing diffuse bilateral parotid lesions. (C,D) LSGB revealing multifocal lymphocytic infiltration between lobules, dilated interlobular ducts, and occasional destruction of the gland. LSGB, labial salivary gland biopsy.
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case_images: PMC9807900_02_fped-10-1077324-g003.jpg
case_images: F3
case_images: Ultrasound showed right parotid enlargement and diffused bilateral parotid lesions (Figures 3A,B). Labial salivary gland biopsy (LSGB) revealed multifocal lymphocytic infiltration between the lobules, dilated interlobular ducts, and occasional destruction of the gland (Figures 3C,D).
case_images: Clinical course of the patient with APDS1. APDS1, activated phosphoinositide 3-kinase-delta syndrome 1.
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case_images: PMC9807900_02_fped-10-1077324-g004.jpg
case_images: F4
case_images: Therefore, in addition to sirolimus, prednisone, mycophenolate mofetil (MMF), and hydroxychloroquine (HCQ) were administered (Figure 4). Intermittent bilateral parotid pain recurred in March 2021 and was not relieved until the prednisone dosage was increased to 20 mg/day in July 2021 (Figure 4).