Coincidence of Systemic Lupus Erythematosus and ANCA-Associated Vasculitis: A Case Report with Perforation of Nasal Septum and Palate

Figures & data

Table 1 Laboratory Results of First Admission

Item	Actual Value	Normal Value
Complete blood count
WBC	4.89 × 10^9/L	(3.50–9.50) × 10^9/L
Neutrophils	3.26 × 10^9/L	(1.80–6.30) × 10^9/L
Lymphocyte	1.03 × 10^9/L	(1.10–3.20) × 10^9/L
RBC	4.14 × 10^9/L	(3.80–5.10) × 10^9/L
Hemoglobin	122 g/L	(115–150) g/L
Hematocrit	37.5%	(35.0–45.0)%
MCV	90.6 fL	(82–100) fL
Platelet	136 × 10^9/L	(125–350) × 10^9/L
ESR	65 mm/h	(0–20) mm/h
CRP	1.28 mg/L	(0–8) mg/L
Biochemistry
CK	21 U/L	(40–200) U/L
LDH	221 U/L	(120–250) U/L
AST	74 U/L	(5–40) U/L
ALT	79 U/L	(0–55) U/L
ALP	81 U/L	<187 U/L
GGT	86 U/L	(7–45) U/L
TBIL	9.9 umol/L	(0–23.0) umol/L
DBIL	4.5 umol/L	(0–6.8) umol/L
UBIL	5.4 umol/L	(0–13.7) umol/L
TP	77.7 g/L	(65–85) g/L
Alb	37.4 g/L	(35–55) g/L
Glb	40.3 g/L	(20–30) g/L
BUN	5.6 mmol/L	(2.9–7.9) mmol/L
Creatinine	43 umol/L	(41–81) umol/L
eGFR	146.1 mL/min/1.73 m^2
Immunology
ANA	1:80 (speckled)	<1:40
Anti-dsDNA Ab	(-)	(-)
Anti-Sm Ab	(+)	(-)
Anti--U1-RNP Ab	(+)	(-)
Anti-SS-A Ab	(+)	(-)
Anti-SS-B Ab	(+)	(-)
Anti-P Ab	(+)	(-)
MPO-ANCA Ab	<2.00 RU/mL	<20.00 RU/mL
PR3-ANCA Ab	<2.00 RU/mL	<20.00 RU/mL
Anti-GBM Ab	<2.00 RU/mL	<20.00 RU/mL
Anti-AMA Ab	(-)	(-)
Anti-gp210 Ab	(-)	(-)
Anti-SP100 Ab	(-)	(-)
C3	0.39 g/L	(0.79–1.52) g/L
C4	<0.0167 g/L	(0.16–0.38) g/L
IgM	1.54 g/L	(0.46–3.04) g/L
IgG	22.20 g/L	(7.51–15.60) g/L
IgA	2.93 g/L	(0.82–4.53) g/L
Urinalysis
Urine protein	(±)	(-)
24-hour urine protein	337 mg/24h urine	(0–300) mg/24h urine
Occult blood	(-)	(-)
Urine WBC	(-)	(-)
Urine RBC	(-)	(-)

Table 2 Laboratory Results of Second Admission

Item	Actual Value	Normal Value
Complete blood count
WBC	1.30 × 10^9/L	(4.1–11.0) × 10^9/L
Neutrophils	0.09 × 10^9/L	(1.80–8.30) × 10^9/L
Lymphocyte	0.91 × 10^9/L	(1.10–3.20) × 10^9/L
RBC	2.82 × 10^9/L	(4.10–5.30) × 10^9/L
Hemoglobin	82 g/L	(114–154) g/L
Hematocrit	25.1%	(36.0–47.0)%
MCV	89.0 fL	(80–100) fL
Platelet	44 × 10^9/L	(150–407) × 10^9/L
ESR	62 mm/h	(0–20) mm/h
CRP	1.57 mg/L	(0–8) mg/L
Biochemistry
CK	208 U/L	(40–200) U/L
LDH	988 U/L	(120–250) U/L
AST	215 U/L	(5–40) U/L
ALT	42 U/L	(0–55) U/L
ALP	76 U/L	<187 U/L
GGT	75 U/L	(7–45) U/L
TBIL	12.4 umol/L	(0–23.0) umol/L
DBIL	6.8 umol/L	(0–6.8) umol/L
UBIL	5.6 umol/L	(0–13.7) umol/L
TP	60.6 g/L	(65–85) g/L
Alb	25.0 g/L	(35–55) g/L
Glb	35.5 g/L	(20–30) g/L
BUN	6.3 mmol/L	(2.9–7.9) mmol/L
Creatinine	31 umol/L	(41–81) umol/L
eGFR	162.7 mL/min/1.73 m^2
Immunology
ANA	1:80 (speckled)	<1:40
Anti-dsDNA Ab	(-)	(-)
Anti-Sm Ab	(-)	(-)
Anti--U1-RNP Ab	(+)	(-)
Anti-SS-A Ab	(+)	(-)
Anti-SS-B Ab	(+)	(-)
Anti-P Ab	(+)	(-)
MPO-ANCA Ab	<2.00 RU/mL	<20.00 RU/mL
PR3-ANCA Ab	>400.00 RU/mL	<20.00 RU/mL
Anti-GBM Ab	<2.00 RU/mL	<20.00 RU/mL
Anti-AMA Ab	(-)	(-)
Anti-gp210 Ab	(-)	(-)
Anti-SP100 Ab	(-)	(-)
C3	0.24 g/L	(0.79–1.52) g/L
C4	<0.0167 g/L	(0.16–0.38) g/L
IgM	0.95 g/L	(0.46–3.04) g/L
IgG	26.70 g/L	(7.51–15.60) g/L
IgA	0.91 g/L	(0.82–4.53) g/L
Urinalysis
Urine protein	(1+)	(-)
24-hour urine protein	604 mg/24h urine	(0–300) mg/24h urine
Occult blood	(-)	(-)
Urine WBC	(-)	(-)
Urine RBC	(-)	(-)

Figure 1 Perforation of the hard palate, and the nasal cavity could be peered through the perforation.

Figure 2 CT showed the palatal perforation (red arrow).

Figure 3 The nasal endoscopic biopsy section indicates inflammatory polyp with chronic suppurative inflammation and inflammatory granulomatous hyperplasia. (Hematoxylin-eosin stain).

Figure 4 Immunohistochemical tests of nasal biopsy were negative. (A) EBER. (B) Ki-67. (C) CD19. (D) CD20. (E) TIA-1. (F) CD8. (G) CD3. (H) CD2. (I) CD56. (J) CD79a. (K) CD7. (L) CD4. (M) Granzyme B. (N) Pax-5.

Table 3 Summary of Patients with the SLE/AAV Syndrome from Previous Reports in the Literature

	Ratio
Sex ratio, (F/M)	18/0
Mean age at diagnosis of SLE/AAV overlap(y±SD)	42±18
Manifestations of SLE/AAV overlap
Nondestructive polyarthritis	83.33%(15/18)
Hematology
Thrombopenia	66.67%(12/18)
Lymphopenia	16.67%(3/18)
Anemia	38.89%(7/18)
Nervous System
Subarachnoid hemorrhage	5.56%(1/18)
Cerebral ischemia	11.11%(2/18)
Respiratory System
Pulmonary nodules	11.11%(2/18)
Pleural effusion	5.56%(1/18)
Pulmonary inflammatory	5.56%(1/18)
Intra-alveolar hemorrhage	11.11%(2/18)
Skin Mucosa
Malar rash	16.67%(3/18)
Skin nodules	5.56%(1/18)
Discoid lupus erythematosus	16.67%(3/18)
Nasal involvement	27.78%(5/18)
Cardiac involvement	16.67%(3/18)
Renal involvement
Nephrotic syndrome	5.56%(1/18)
RPGN (CGN)	16.67%(3/18)
Acute renal failure	5.56%(1/18)
Anti-MPO	100%(18/18)