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 13, Number 6, June 2022, pages 290-296

A Continuous Increase in CXC-Motif Chemokine Ligand 10 in a Case of Anti-Nuclear Matrix Protein-2-Positive Juvenile Dermatomyositis

Figures

Figure 1. Skin manifestations. (a) Heliotrope rash, (b) Gottron’s papules and (c) erythema over the knees. (d) Magnetic resonance imaging showing T2-weighted and short tau inversion recovery (STIR) images of the lower limbs.

Figure 2. Muscle biopsy findings. (a) Hematoxylin and eosin staining showing perifascicular atrophy with atrophic basophilic fibers at the periphery of the fascicles in addition to minimal perivascular infiltration of lymphocytes. Immunohistochemical staining for (b) human leukocyte antigen (HLA)-ABC, (c) myxovirus-resistance protein A (MxA) showing the expression of interferon-induced protein, and (d) membrane attack complex (MAC) showing complement deposition in the endomysial capillaries.

Figure 3. (a) Cytokine profiling of healthy adults (n = 8), a patient with systemic juvenile idiopathic arthritis (sJIA) (n = 1, four samples obtained before treatment initiation), patients with Kawasaki disease (n = 6), and this patient (two samples obtained before treatment initiation, and a further 16 consecutive samples). (b) Clinical course of the patient. Content of treatment, sequential changes in serum creatine kinase (CK, IU/L), aldolase (IU/L), soluble interleukin-2 receptor (sIL-2R, IU/L) beta-2 microglobulin (β2MG, µg/dL) levels, as well as retrospectively evaluated serum CXC-motif chemokine ligand (CXCL)-10 levels (pg/mL) are shown. The gray arrows on the vertical axis indicate the upper limits of Aldorase, sIL-2R, and β2MG, as well as the average CXCL-10 in healthy adults.

Table

**Table 1.** Laboratory Results
WBC: white blood cell count; RBC: red blood cell count; Hb: hemoglobin; PLT: platelets; PT-INR: prothrombin time-international normalized ratio; APTT: activated partial thromboplastin time; Fib: fibrinogen; AT III: antithrombin III; ds: double-stranded; Sm: Smith; SS: Sjogren’s syndrome; CCP: cyclic citrullinated peptide; RNP: ribonucleoprotein; TP: total protein; AST: aspartate aminotransferase; ALT: alanine aminotransferase; LDH: lactate dehydrogenase; CK: creatine kinase; BUN: blood urea nitrogen; NXP: nuclear matrix protein; ARS: aminoacyl-tRNA synthetases; MDA5: melanoma differentiation associated gene-5; Ig: immunoglobulin; sIL-2R: soluble interleukin-2 receptor; TIF1γ: transcriptional intermediary factor 1γ; CRP: C-reactive protein; ESR: erythrocyte sedimentation rate; β2MG: β2-microglobulin; NTproBNP: N-terminal proB-type natriuretic peptide.
WBC	5,450/µL	TP	8.4 g/dL	CRP	0.38 mg/dL
Neutrophils	3,270/µL	Albumin	3.3 g/dL	ESR - 1 h	45 mm
Lymphocytes	1,800/µL	AST	71 IU/L	ESR - 2 h	61 mm
RBC	4,370 × 10³/µL	ALT	20 IU/L	Ferritin	373.9 ng/dL
Hb	11.0 g/dL	LDH	912 IU/L	Serum β2MG	4.77 µg/mL
PLT	421 × 10³/µL	CK	136 IU/L	Urine β2MG	4.55 µg/L
		Aldolase	33.4 IU/L	sIL-2R	3,338 U/mL
PT-INR	1.03	Amylase	67 IU/L	IgG	3354.7 mg/dL
APTT	36.8 s	BUN	11.6 mg/dL	IgA	334.1 mg/dL
Fib	250 mg/dL	Creatine	0.22 mg/dL	IgM	66.1 mg/dL
D-dimer	5.7 mg/dL	Na	137 mEq/L	KL-6	258 U/mL
AT III	81%	K	4.2 mEq/L	NTproBNP	337.4 pg/mL
		Cl	99 mEq/L
Anti-nuclear antibody	1:640	Anti-NXP2 antibody	+
Anti-dsDNA antibody	-	Anti-Jo-1 antibody	-
Anti-Sm antibody	-	Anti-ARS antibody	-
Anti-SS-A antibody	-	Anti-TIF1γ antibody	-
Anti-SS-B antibody	-	Anti-MDA5 antibody	-
Anti-CCP antibody	0.8 U/mL	Anti-Mi-2 antibody	-
Anti-U1-RNP antibody	-