Highlighting the importance of early diagnosis in progressive multi-organ involvement of IgG4-related disease: A case report and review of literature

Table 1 Main laboratory work-up, imaging and histology studies from 2000 to 2018

Time	Investigations	Results
February 10, 2000	Physical examination	Non-specific, non-infective orbital inflammatory pseudotumor
July 9, 2003	CT	Enlargement of double lacrimal glands, parotid glands
	Lacrimal glands biopsy	Lymphonid pseudotumor of double lacrimal glands
May 21, 2005	CT	Enlargement of parotid glands
	B-ultrasonography	Diffused enlargement of parotid glands
November 2, 2005	Blood routine examination	LYM%: 50.9% (20.0-50.0), LYM#: 2.11 × 10-9/L (1.10-3.20), EOS%: 13.6% (0.4-8.0), EOS#: 0.78 × 10-9/L (0.02-0.50), NEUT%: 68.0% (50.0-70.0), NEUT#: 6.62 × 10-9/L (2.00-7.00)
	Biochemistry	AST: 628.5 U/L (13.0-35.0), ALT: 648.4 U/L (7.0-40.0), ALP: 356 U/L (50-135), GGT: 422.9 U/L (7.0-45.0)
	CT	Enlargement of the head of pancreas
December 4, 2005	ERCP	Implantation of a biliary stent
March 6, 2006	IGG, RF	High levels of serum IgG, RF, Ig light chain KAP, Ig light chain LAM
March 31, 2006	ERCP	Implantation of two biliary stents
	Biopsy	Extensive lymphatic plasma cell infiltrated in focal lobular, salivary gland tissue was under the squamous mucosa
July 8, 2006	IGG, RF	Low levels of serum complement C3 and IgG and Ig light chain KAP, high level of Ig light chain LAM
November 4, 2006	Complement	Low level of serum complement C3
	ENA antibody, ANA	HEp2-ANA: positive 1:100 (< 1;100), ENA-AbSSA: negative, ENA-AbSSB: negative
November 8, 2006	ERCP	Remove of the biliary stent
November 26, 2006	CT	No enlargement of the head of pancreas
	Physical examination	Normal eyeball, normal conjunctiva, eyelids without edema, normal light reaction
September 4, 2007	Blood routine examination	Normal parameters
September 6, 2007	Endocrine examinations	Details seen in Table 2
October 10, 2007	Renal function examination	Normal function
May 17, 2008	MRI	Pituitary stalk thickening
October 7, 2009	CT	Enlargement of the head of pancreas
November 13, 2009	Blood routine examination	LYM%: 46.0% (20.0-50.0), LYM#: 1.92 × 10-9/L (1.10-3.20), EOS%: 23.1% (0.4-8.0), EOS#: 0.89 × 10-9/L (0.02-0.50), NEUT%: 49.0% (50.0-70.0), NEUT#: 5.83 × 10-9/L (2.00-7.00)
December 14, 2009	MRI	Enlargement of the head of pancreas
September 20, 2011	CT	Normal volume of the head of pancreas
November 22, 2011	Bone scan	Rib fracture
November 23, 2011	Blood routine examination	LYM%: 22.6% (20.0-50.0), LYM#: 1.85 × 10-9/L (1.10-3.20), EOS%: 11.1.% (0.4-8.0), EOS#: 0.97 × 10-9/L (0.02-0.50), NEUT%: 59.0% (50.0-70.0), NEUT#: 4.83 × 10-9/L (2.00-7.00)
	IGG, RF	High levels of IgE, RF
November 24, 2011	IGG	IgG1: 7.670 g/L (4.900-11.400 ), IgG2: 3.540 g/L (1.500-6.400), IgG3: 0.103 g/L (0.200-1.100), IgG4: 8.650 g/L (0.020-2.000)
	IGG, RF	High levels of serum IgE and RF
July 5, 2012	Blood routine examination	LYM%: 38.9% (20.0-50.0), LYM#: 3.99 × 10-9/L (1.10-3.20), EOS%: 1.6% (0.4-8.0), EOS#: 0.16 × 10-9/L (0.02-0.50), NEUT%: 51.8% (50.0-70.0), NEUT#: 5.32 × 10-9/L (2.00-7.00)
July 10, 2012	MRI	Normal shapes of pituitary gland, lung and pancreas
April 3, 2018	Blood routine examination	EOS%: 0.0% (0.4-8.0), EOS#: 0.00 × 10-9/L (0.02-0.50), LYM%: 15.0% (20.0-50.0), LYM#: 1.72 × 10-9/L (1.10-3.20), NEUT%: 82.6% (50.0-70.0), NEUT#: 9.44 × 10-9/L (2.00-7.00)
	Urinalysis	Normal, Only the WBC count 26.3/uL (0.0-23.0 )
	Biochemistry	UREA: 2.10 mmol/L (3.10-8.80), ALB: 36.10 g/L (40.00-55.00), AST: 43.6U/L (13.0-35.0), ALP: 43U/L (50-135), GGT: 5.1 U/L (7.0-45.0)
	IGG, Complement, RF	IgG: 25.10 g/L (7.00-16.00), IgA:1.51 g/L (0.70-4.00), IgM: 0.45 g/L (0.40-2.30g/l), C3: 0.56 g/L (0.90-1.80), C4: 0.088 g/L (0.100-0.400), RF: 356.00 IU/mL (0.00-19.00), IgG4: 23.300 g/L (0.020-2.000)
	CT	Severe interstitial lung lesions
	Bladder biopsy	Chronic inflammation
May 7,2018	IGG	IgG4: 5.280 g/L (0.020-2.000)
	CT	Normal

ANA-HEp-2: Anti-Nuclear Antibodies HEp-2; CT: Computed tomography; ERCP: Endoscopic retrograde cholangio pancreatography; ENA: Extractable nuclear antigens; NEUT: Neutrophil count; AST: Aspartate transferase; ALT: Alanine aminotransferase; ALP: Alkaline phosphatase; GGT: Gamma glutamyl transpeptidase; Ig: Immunoglobulin; KAP: Kappa; LAM: Lambda; MRI: Nuclear magnetic resonance image; RF: Rheumatoid factor; WBC: White blood cell; RBC: Red blood cell; EOS: Eosinophils; LYM: Lymphocyte; LYM#: The absolute value of lymphocyte; PCT: Platelet distributing width; ESR: Erythrocyte sedimentation rate; ALB: Albumin; IgG: Immunoglobulin G; IgA: Immunoglobulin A; IgM: Immunoglobulin M; C3: Complement 3; C4: Complement 4.

Table 2 Endocrine examinations in September 2007

Date	Serum osmotic pressure (270-300 mOsm/L)			Urine osmotic pressure (400-1200 mOsm/L)			Urine NAG enzyme (0-21 U/gCr)
September 24	289 mOsm/L			62 mOsm/L			-
September 25	298 mOsm/L			65 mOsm/L			87.5 U/gCr
September 28	300 mOsm/L			148 mOsm/L			106.5 U/gCr
	Serum prolactin (PRL) (µg/L)
September 30	Pre-stimulation with metoclopramide (10 mg)				Post-stimulation with metoclopramide (10 mg)
	15 min		0 min		15 min	30 min		60 min	90 min
	28.66		29.28		38.78	35.22		38.66	39.84
September 30	Changes of serum hormone levels
	Testosterone	Estrogen	LH	PRL	FSH	Progestone		Cortisol
	-	-	↓	↑	↓	-		12:00 AM	8:00 AM
								-	-

FSH: Follicle stimulating hormone; LH: Luteinizing hormone; NAG: N-acetyl-β-D-glucosaminidase; PRL: Prolacin.