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Table 1 Patient demographics, current antirheumatic therapy, reasons for initiation of subcutaneous immune globulins, and disease course following fSCIg therapy

From: Treatment with high-dose recombinant human hyaluronidase-facilitated subcutaneous immune globulins in patients with juvenile dermatomyositis who are intolerant to intravenous immune globulins: a report of 5 cases

Patient

Sex, age, age at disease onset, body weight

Clinical phenotype and disease severitya, age at disease onset, MSA status

Disease activity at time of fSCIg initiation

Previous or concurrent antirheumatic treatments

Reason for initiation of fSCIg instead of IVIg, CMAS at time of fSCIg initiation

Disease course following initiation of fSCIg

1

Male, 11 years, 9 years at disease onset, 35 kg

Classic, severe JDM with prominent skin involvement, joint contractures, Anti-Mi-2 positive

Clinically inactive (however, recent recurrence after attempted IVIg discontiuation)

IVIg (60 g/month), fSCIg (70 g/month), PDN 0.125 mg/kg/day, MTX 15 mg/m2/week, HCQ

IVIg adverse effects (headaches, nausea and vomiting), needle phobia

CMAS 51

Within 3 months mild deterioration of CMAS (49 instead of 51); resolution after switching to fSCIg 5 days apart

2

Female, 10 years, 8 years at disease onset, 35 kg

Classic, severe JDM with prominent vaculopathy, dysphagia, cutaneous ulcerations, myocarditis, joint contractures, calcinosis, Anti-TIF-1gamma positive

Residual disease activity, vasculopathy

IVIg (70 g/month), fSCIg (70 g/month), PDN 0.21 mg/kg/day, MMF 1200 mg/m2/d, MTX 15 mg/m2/week, RTX (status post 375 mg/m2 ×4), CYC (status post 6 × 750 mg/m2)

IVIg adverse effects (headaches), difficult peripheral venous access with port-a-cath

CMAS not interpretable due to severity of contractures

Stable mild residual disease activity even after switching to fSCIg 5 days apart

3

Female, 7 years, 5 years at disease onset, 20 kg

Classic, severe JDM with prominent vasculopathy, cutaneous ulcerations, Anti-SRP and anti-MI-2 positive

Clinically inactive (recent recurrence)

IVIg (40 g/month), fSCIg (40 g/month), PDN 0.125 mg/kg/day, MMF 1200 mg/m2/d, MTX 15 mg/m2/week

RTX (status post 375 mg/m2 × 4 [first cycle], 750 mg/m2 × 1 [second cycle])

IVIg adverse effects (headaches, nausea and vomiting), needle phobia

CMAS 52

Maintenance of clinically inactive disease; CMAS 52; fSCIg continued biweekly

4

Male, 12 years, 8 years at disease onset, 35 kg

Classic, moderately severe JDM with nodular dystrophic calcification, anti-NXP-2 positive

Clinically inactive (however, progressive calcinosis)

IVIg (35 g/month), fSCIg (60 g/month) PDN 0.07 mg/kg/day, MMF 1200 mg/m2/d, Colchicin

IVIg adverse effects (headaches, nausea and vomiting), needle phobia, residual disease activity with insufficient IVIg dose

CMAS 52

Maintenance of clinically inactive disease; CMAS 52; calcinosis decreasing; fSCIg continued biweekly

5

Female, 8 years, 5 years at disease onset, 20 kg

Classic, severe JDM with rhadomyolysis, failure to thrive and nephrolithiasis, MSA negative

Active skin and muscle disease (IVIg was discontinued 6 months earlier)

IVIG (40 g/month), fSCIg (50 g/month)

PDN 0.19 mg/kg/day, MMF 600 mg/m2/d (diarrhea with higher doses), MTX 15 mg/m2/week

IVIg adverse effects (headaches, nausea and vomiting), needle phobia

CMAS 46

Improvement but residual, mild proximal muscle weakness; normal muscle strength (inactive disease) after switching to fSCIg 5 days apart; CMAS 50; further PDN reduction

  1. Abbreviations: CYC cyclophosphamide, IVIg intravenous immune globulins, fSCIg subcutaneous immune globulins facilitated by recombinant human hyaluronidase, MMF mycophenolate mofetil, MSA myositis-specific antibodies, MTX methotrexate, PDN prednisolone, RTX rituximab
  2. aDefinition of disease severity according to Huber et al. [5]