Case Report

Nephrotic Syndrome as an Extramuscular Manifestation of Anti-EJ Antibody-Positive Dermatomyositis: A Case Report and Review of the Literature

Table 1

Laboratory values on second admission of a woman with limited cutaneous systemic sclerosis who developed nephrotic syndrome in the setting of recurrence of anti-glycyl-transfer ribonucleic acid synthetase antibody (anti-EJ)-positive dermatomyositis.

Laboratory testResultReference range

CBC
WBC count (/μL)10,5002,700–8,800
Lymphocyte (%)16.619–47
RBC count (/μL)458370–540
Hemoglobin (g/dL)12.611.0–17.0
Hematocrit (%)39.034.0–49.0
Platelets (×104/μL)40.814–34

Coagulation
PT-INR1.12
APTT (sec)33.130.0 ± 5.0

Biochemistry
Total protein (g/dL)7.06.6–8.2
Albumin (g/dL)2.03.9–4.9
AST (U/L)388–38
ALT (U/L)274–44
LDH (U/L)440106–211
γGTP (U/L)916–73
Alkaline phosphatase (U/L)191104–338
Total bilirubin (mg/dL)0.500.2–1.2
BUN (mg/dL)17.38.0–22.6
Creatinine (mg/dL)0.560.4–0.8
CK (U/L)116343–165
ESR (mm/hour)19.0<15
CRP (mg/dL)2.09<0.3
KL-6 (U/mL)1163<430
SP-A (ng/mL)124.2<43.8
SP-D (ng/mL)202<110

Urinalysis
Proteinuria≥300 mg/dL(−)
WBC (/HPF)1–4<1–4
RBC (/HPF)1–4<1–4
Casts (/HPF)Hyaline casts ≥ 100
Epithelial casts 1–4
Fatty casts 1–4
(−)

Immunological
IgG (mg/dL)2,623870–1,700
IgA (mg/dL)412110–410
IgM (mg/dL)27335–220
CH50 (U/mL)4725–50
C3 (mg/dL)8065–135
C4 (mg/dL)1513–35
ANA1 : 320 speckled
1 : 40 cytoplasmic
<1 : 40
Anti‐dsDNA IgG Ab (IU/mL)<10<12
Anti‐SSA AbPositive at a titer of 1 : 16Negative
Anti‐SSB AbPositive at a titer of 1 : 2Negative
Anti‐RNP AbPositive at a titer of 1 : 16Negative
Anti‐Sm AbNegativeNegative
Anti-ARS Ab (U/mL)174.0<25.0
Anti-EJ AbPositiveNegative
LAC0.83≤1.16
Ant-cardiolipin Ab (U/mL)≤8<10
Anti-β2GPI Ab (U/mL)≤1.2<3.5
PR3-ANCA (U/mL)<1.0<3.5
MPO-ANCA (U/mL)<1.0<3.5

CBC: complete blood count; WBC: white blood cell; RBC: red blood cell; PT-INR: prothrombin time-international normalized ratio; APTT: activated partial thromboplastin time; sec: seconds; AST: aspartate aminotransferase; ALT: alanine aminotransferase; LDH: lactate dehydrogenase; γGTP: γglutamyl transpeptidase; BUN: blood urea nitrogen; CK; creatine kinase; ESR: erythrocyte sedimentation rate; CRP: C-reactive protein; KL-6: krebs von den lungen-6; SP: surfactant protein; HPF: high power field; IgG; immunoglobulin G; IgM; immunoglobulin M; IgA; immunoglobulin A; CH50; 50% hemolytic complement activity; C3: complement 3; C4: complement 4; ANA: antinuclear antibody; dsDNA: double-stranded deoxyribonucleic acid; Ab: antibody; SSA: Sjögren’s syndrome-related antigen A; SSB: Sjögren’s syndrome type B; RNP: ribonucleoprotein; Sm: Smith; ARS: aminoacyl-transfer ribonucleic acid (tRNA) synthetase; EJ: glycyl-transfer ribonucleic acid synthetase; LAC: lupus anticoagulant; β2GPI: β2-glycoprotein; PR3: proteinase 3; MPO: myeloperoxidase; ANCA: anti-neutrophil cytoplasmic antibody.