Table 1 Clinical, laboratory and radiological findings in two IgG4-RD patients

IgG4-related clinical symptoms

AKI, AKD on CKD

AKD

Ileus

Asthma, rhinitis

Fatigue

Fatigue, weight loss

IgG4-RD involved organs, identified by

 Clinical presentation/enhanced-CT/US (measured by US, cm³)

Kidneys (13.0 × 6.3 × 5.8/12.2 × 5.9 × 4.5)

Kidneys (13.0 × 7.5 × 6.4/13.3 × 6.6 × 5.7)

Submaxillary glands

Submaxillary glands

Abdominal aortitis

–

Superior mesenteric, splenic arteritis

–

Pathology

Kidney (right)

Kidney (right)

Bone marrow

ND

FDG-PET/CT (SUV max)

Kidneys (5.0)

Kidneys (3.7)

Submaxillary glands (3.0)

Submaxillary glands (2.9)

Hilar, mediastinal lymph nodes (4.0)

Hilar, mediastinal lymph node (3.5)

–

Bone marrow (3.6)

Number of involved sites

6

6

Laboratory data

 WBC (3.5–9.5 × 10⁹/L)

3.4

4.2

  NE (40.0–75.0%)

60.8

54.8

  LY (20.0–50.0%)

27.2

18.8

  EO (0.4–8.0%)

4.0

15.0

 Platelet count (125–350 × 10⁹/L)

110

173

 Hemoglobin (130–175 g/L)

108

122

 Total protein (65–85 g/L)

73.1

99.5

 Albumin (40–55 g/L)

34.3

36.7

 BUN (1.8–7.1 mmol/L)

17.27

7.2

 Creatinine (44–133 μmol/L)

326

146

 Amylase (35–135 IU/L)

219

ND

 C3 (0.6–1.5 g/L)

0.519

0.486

 C4 (0.12–0.36 g/L)

0.15

0.113

 IgG (7.23–16.85 g/L)

29.56

64.77

  IgG1 (4.90–11.40 g/L)

8.35

12.60

  IgG2 (1.69–7.86 g/L)

8.01

3.57

  IgG3 (0.11–0.86 g/L)

1.50

1.40

  IgG4 (0.03–2.01 g/L)

11.70

47.20

 IgA (0.69–3.82 g/L)

2.33

1.01

 IgM (0.63–2.77 g/L)

0.50

0.57

 IgE (< 100 kU/L)

ND

1761

 hs-CRP (0.0–3.0 mg/L)

8.56

9.99

 ESR (0–15 mm/h)

52

92

 Urinary protein (0–0.15 g/day)

0.53

0.41

 ANA

1:3200

 < 1:100

 dsDNA (IU/mL)

262

 < 1:10

 Lymphocytes T CD4⁺(34.0–70.0%)

ND

38.26

 Lymphocytes T CD8⁺(14.0–41.0%)

ND

57.46