| | Ref. no. | Sex/age | Preceding manifestations | Previous treatment | Admission/after RTX [maximum1] | RTX targeting lesion | Duration prior to RTX2 | RTX dosage (cycles) | Therapy during or after RTX | Outcome | | Respiratory (ILD) | Muscular (CK levels) | Cutaneous | Others | Ferritin (ng/mL) | KL-6 (U/mL) |
| | [14] | F/68 | Cough, unusual dyspnea | Generalized weakness (normal) | Raynaud, erythema on the face, back, limbs, and hands | Fever, arthralgia, appetite loss | mPSL, PSL, IVIg, IVCY, MMF, CsA, Tac (topical), HCQ | 805/n.d. | n.d./n.d. | Cutaneous lesion | About 2 years | 1000 mg, per 15 days (×2) | n.d. | Improved3 | | [15] | F/58 | n.d. | Weakness on the deltoids (normal) | Heliotrope rash, Gottron’s papules | n.d. | PSL, Tac, IVCY | 891.7/n.d. | 613/2756 [4185] | ILD | About 3 months | 500 mg, 375 mg/m2/week (×4) | mPSL, PSL, IVCY, IVIg, PMX | Improved | | [16] | F/55 | Rapidly progressive shortness of breath | None (normal) | Raynaud, heliotrope rash, Gottron’s papules, rash on the hands | Weight loss | mPSL | n.d./n.d. | n.d./n.d. | ILD | n.d. | n.d. | CPA, PE | Died | | [17] | F/71 | Rapid deterioration of respiratory status, hypoxia | None (211 U/L) | Heliotrope rash, Gottron’s papules, ulcer on the buttocks, papules on the fingers and elbows | Appetite loss, fatigue | mPSL, PSL, IVCY, IVIg, PMX | 1782.8/253.1 [3149.8] | 666/4 | ILD cutaneous lesion | 102 days | 525 mg, 350 mg/m2/week (×4) | PSL, Tac | Improved | | [18] | F/71 | Dry cough, continuous deterioration of respiratory status | None (n.d.) | Purpura on the elbows, erythema on the anterior chest | Fever | mPSL, PSL, Tac, CsA, IVCY | 507/1740 [1740] | 991/n.d. | ILD | 38 days | 600 mg, 375 mg/m2/week (×2) | mPSL, PSL, CsA, MMF, Tac | Died | | [18] | F/69 | Exertional dyspnea, respiratory distress with hypoxia | None (225 U/L) | Gottron’s papules, rash on the extremities, hyperkeratosis on the palmer side of fingers | Arthralgia | mPSL, PSL, CsA | 219/1930 | 922/1520 | ILD | 33 days | 500 mg, 375 mg/m2/week (×2) | mPSL, PSL, CsA, IVCY, tocilizumab, CHD | Died | | This case | M/48 | Exertional dyspnea with hypoxia, dry cough | Mild weakness on the lower limbs (278 U/L) | Heliotrope rash, Gottron’s papules, mechanic’s hands, palmar papules, erythema on the face and back, ulcer/erosion on the elbows | Fatigue, fever, appetite loss, arthralgia | mPSL, PSL, CsA, IVCY | 781/186 [1437] | 602/638 [1674] | ILD cutaneous lesion | 125 days | 700 mg, 375 mg/m2/week (×4) | PSL, CsA | Improved |
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DM, dermatomyositis; ILD, interstitial lung disease; Ref., reference; CK, creatine kinase; n.d., not described; Raynaud, Raynaud phenomenon; RTX, rituximab; mPSL, methylprednisolone; PSL, prednisolone; IVIg, intravenous immunoglobulin; IVCY, intravenous cyclophosphamide; MMF, mycophenolate mofetil; CsA, cyclosporine A; Tac, tacrolimus; HCQ, hydroxychloroquine; CPA, cyclophosphamide; PMX, polymyxin B hemoperfusion treatment; PE, plasma exchange; CHD, continuous hemodiafiltration. 1Maximum value if it was described in the report. 2Duration prior to administering RTX since initiating hospitalization. 3Remission of painful erythematous papules on the hands was obtained [14]. 4Decrease of KL-6 levels after RTX administration was shown in the figure of the described report [17].
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