Table 1.
Features of juvenile myositis patients with PJP infection
| Pt | Sex | Race | Age at JM dx (years) | MSAa | Clinical featuresb | JM dx to PJP dx (months) | Prednisone mg; mg/kgc | IVMP (pulses)c | IVIGd | MTXd | HCQd | MMF or CSAd | CYC (weeks)d | RIT (weeks)d | ALC ×109/l | Age of death (years) | ICU (days) | ||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| 1 | F | Asian | 16.0 | MDA5 | ILD, U, DI | 27.9 | 60; 1.1 | X (1) | X | X | X | X (4) | X (4) | 0.59 | 4 | ||||
| 2 | M | Nat Am | 1.6 | MDA5 | ILD, U, DI | 1.8 | 12.5; 1.0 | X (na) | X | 2.20 | |||||||||
| 3 | F | AA | 2.0 | MDA5 | ILD | 0.3 | 20; 2.0 | X (>4) | X (0) | 2.10 | 2.1 | 16 | |||||||
| 4 | F | His | 2.4 | MDA5 | U | 1.8 | 24; 1.8 | X | X | 6.85 | 2.9 | 150 | |||||||
| 5 | F | Asian | 17.2 | MDA5 | U | 1.6 | 60; 1.0 | X (2) | X | X (0) | 0.76 | ||||||||
| 6 | F | C | 14.6 | Nonee | U | 5.1 | 40; 1.1 | X (3) | X | X | X | X (20) | 1.57 | 1 | |||||
| 7 | M | C | 2.9 | Nonef | U | 2.0 | 30; 1.7 | X | X | X | 1.87 | ||||||||
| 8 | M | C | 9.2 | Nonef | U, DI | 13.2 | 30; 0.8 | X | X | X | X (20) | 0.72 | |||||||
| 9g | M | AA, C | 9.5 | Nonee | None | 2.3 | 30; 0.9 | X | 2.10 | 9.8 | 111 | ||||||||
| 10 | F | AA | 6.2 | Nonee | None | 50.8 | 30; 0.8 | X (4) | X | X | 2.27 | ||||||||
| 11 | F | C | 5.5 | NT | None | 6.6 | 20; 0.7 | X (>4) | X | X | X | 1.73 | |||||||
| 12 | M | C | 2.3 | NT | None | 1.0 | 30; 2.0 | X (>4) | X | X | 6.41 | 2.5 | 52 | ||||||
| 13 | M | C | 3.1 | p155/140 (TIF1) | None | 7.0 | 12.5; 0.8 | X | X | 0.11 | 28 | ||||||||
Bold text indicates ALC below normal range for age.
MSA testing completed at Oklahoma Medical Research Foundation (five), RDL Laboratory (three), Advance Diagnostics Laboratory (two) and ARUP Laboratory (one).
Clinical features included: ILD, skin ulcerations, digital infarcts.
Medication was received within the month prior to PJP onset; pulses: number of pulses the month prior to PJP.
Medication was received within the 6 months prior to PJP onset; weeks: weeks elapsed since last dose.
MSA testing did not include anti-HMGCR autoantibodies.
MSA testing did not include anti-MDA5, anti-p155/140 (TIF1), anti-NXP2 or anti-HMGCR autoantibodies.
Juvenile polymyositis. PJP: Pneumocystis jirovecii pneumonia; Pt: patient; F: female; M: male; His: Hispanic; AA: African American; C: Caucasian; Nat Am: Native American; JM: juvenile myositis; dx: diagnosis; MSA: myositis-specific autoantibody; MDA5: melanoma differentiation associated protein 5; NT: not tested; TIF1: transcription intermediary factor 1; NXP2: nuclear matrix protein-2; ILD: interstitial lung disease; U: skin ulcerations; DI: digital infarcts; IVMP: intravenous methylprednisolone; na: not answered; CSA: ciclosporin; RIT: rituximab; ALC: absolute lymphocyte count; ICU: intensive care unit.