J Med Cases

Journal of Medical Cases, ISSN 1923-4155 print, 1923-4163 online, Open Access

Article copyright, the authors; Journal compilation copyright, J Med Cases and Elmer Press Inc

Journal website https://www.journalmc.org

Case Report

Volume 12, Number 3, March 2021, pages 107-111

Immunoglobulin G4 Related-Disease: A Rare Presentation With Secondary Hypereosinophilic Syndrome and Eosinophilic Ascites

Figures

Figure 1. Evolution of the peripheral blood absolute eosinophil count (normal range (light-gray): 40 - 400 cells/µL) during the disease course. Day 0 refers to admission day. The patient started corticosteroids on day 2 and was discharged on day 10. Thereafter, the patient was observed as an outpatient on days 13, 36, and 94, remaining stable despite progressive corticosteroid tapering.

Figure 2. Upper digestive tract of the young female patient 2 days after starting the steroid therapy. In the superficial esophagus biopsy fragments obtained in the upper digestive tract endoscopy there was a light inflammatory infiltrate composed mainly by eosinophils, recognizable by their cytoplasmic granules that appear brick-red after eosin staining (yellow circles) with a maximum of five eosinophils per high-power field. (hematoxylin and eosin stain (H&E), × 200 magnification).

Figure 3. Lower digestive tract of the young female patient 2 days after starting methylprednisolone. In the ileal biopsy fragments obtained in the lower digestive tract endoscopy the global architecture and mucosecretory activity were globally preserved, with the chorion containing a mild polymorphic inflammatory infiltrate rich in eosinophils, recognizable by their cytoplasmic granules that appear brick-red after eosin staining (yellow circles) (hematoxylin and eosin stain (H&E), × 200 magnification).

Table

**Table 1.** Immunology and Serology Laboratory Results of the Young Female Patient
	Patient	Normal range
Anti-dsDNA: anti-double stranded DNA; HAV: hepatitis A virus; HB: hepatitis B; HCV: hepatitis C virus; HIV: human immunodeficiency virus; HTLV: human T-cell lymphotropic virus.
Immunoglobulins (Igs)
IgA, mg/dL	150.0	114.0 - 457.0
IgG, mg/dL	1,061.0	793.0 - 1,590.0
IgG1, mg/dL	778.0	240 - 1,118
IgG2, mg/dL	267.0	124 - 549
IgG3, mg/dL	44.2	21 - 134
IgG4, mg/dL	183.0	7 - 89
IgM, mg/dL	30.9	29.0 - 226.0
IgE, KU/L	13	< 100
Ig light-chains kappa, mg/dL	247
Ig light-chains lambda, mg/dL	144
Ratio kappa/lambda	1.72	1.35 - 2.70
Complement (C)
C3, mg/dL	119.5	81.0 - 167.0
C4, mg/dL	12.0	11.0 - 42.0
Allergy
Phadiatop	Negative
Tryptase, µg/L	2.41	< 11.4
Auto-antibodies (Ab)
Anti-nuclear antibodies (ANAs)	Negative
Anti-dsDNA, UI/mL	0.6	< 15.0
Anti-Sm, U/mL	0.2	< 10.0
Anti-SS-A, U/mL	0.2	< 10
Anti-SS-B, U/mL	0.1	< 10
IgA anti-transglutaminase, U/mL	0.3	< 10
Antineutrophil cytoplasmic antibodies (ANCAs)	Negative	< 1/20
Anti-proteinase 3 (PR3), UQ	< 2.3	< 20
Anti-myeloperoxidase (MPO), UQ	4.1	< 20
IgA anti-Saccharomyces cerevisiae antibody (ASCA), U/mL	1.7	< 10
IgG anti-Saccharomyces cerevisiae antibody (ASCA), U/mL	0.4	<10
Serology
Anti-Treponema pallidum Ab	Negative
Anti-HAV Ab	Negative
Antigen HBs	Negative
Anti-HBs Ab, UI/L	Positive (94.1)
Anti-HBc Ab	Negative
Anti-HCV Ab	Negative
Anti-HIV Ab	Negative
Anti-HTLV I	Negative
Anti-HTLV I	Negative
Fasciola hepatica	< 1/160	Negative: < 1/160
Toxocara canis	Negative
Schistosoma	Negative
Echinococcus IgG index	0.7	Negative: < 9
Strongyloides stercoralis Ab	Negative
Ascaris, kUA/L	0.01	< 0.35