Table 1.
Patient characteristics.
| Characteristic | Patient 1 | Patient 2 | Patient 3 | |
|---|---|---|---|---|
| Sex | Female | Female | Female | |
| Age (at the time of IA) | 74 | 55 | 55 | |
| Diagnosis | DM (06/2017) | DM (04/2020) | IMNM (2010) | |
| Clinical presentation | Skin • Gottron papules • Erythema of face and décolleté |
Skin • Angioedema of the face • Heliotrope erythema • Gottron papules • Holster sign • Hypomimia |
Muscle • Proximal muscle weakness |
|
| Muscle • Tetraparesis |
Muscle • Tetraparesis |
Extra-muscular • None |
||
| Lung • None |
Lung • None |
|||
| Other • None |
Other • Dysphagia • Angina pectoris |
|||
| ANA (ICAP pattern) | 1:32,000 (not reported) | 1:1000 (AC-4) | 1:1000 (AC-20) | |
| Myositis-specific antibodies | αMi-2 | αTIF-1γ, Ro-52 | αHMG-CoA-reductase | |
| Histopathological findings in biopsies | Muscle Necrotizing muscular atrophy, sarcolemmal upregulation of MHC-I 15 |
Not performed | Muscle Necrotizing myopathy, upregulation of MHC-I on muscle fibers, phagocytosis by CD4+ cells and macrophages, very few CD4+ and CD8+ cells in between intact muscle fibers |
|
| MITAX (organ system scores) | Before IA | 07/2018: 0.24 (constitutional: 3, cutaneous: 3, muscles: 9) |
06/2020: 0.33 (constitutional: 3, cutaneous: 3, GI: 9, pulmonary: 3, muscles: 3) |
02/2021: 0.19 (constitutional: 3, muscles: 9) |
| After termination of IA | 03/2019: 0.02 (constitutional: 1) |
09/2020: 0.03 (constitutional: 1, muscles: 1) |
10/2021: 0.06 (constitutional: 1, muscles: 3) |
|
| During follow-up | 12/2019: 0.06 (constitutional: 1, cutaneous: 3) |
01/2021: 0.00 | 03/2023: 0.06 (constitutional: 1, muscles: 3) |
|
| Pharmacological therapy | Prednisolone, AZA, IVIG, MTX, RTX | Prednisolone, AZA, RTX, IVIG, MMF, BNB, TOF | Prednisolone, AZA, MTX, RTX, MMF, IVIG, TAC, TOF | |
| IA sessions | 6 sessions (08/2018) | 10 sessions (06–07/2020) | 15 sessions (02–03/2021) | |
| IA-related complications | None | None | None | |
| Intravenous device | Shaldon catheter | Shaldon catheter | Demers catheter | |
| Further course | • Recovery of everyday competence • 01/2020: aggravation of myopathy, increase in CK • Progressive dyspnea due to aortic aneurysm and inoperability; best supportive care • Death of pneumonia (2020) |
• Stable remission of DM • Invasive carcinoma of the breast 01/2021; neoadjuvant chemotherapy with epirubicin, paclitaxel, cyclophosphamide, carboplatin + surgery |
Stable partial remission under ongoing treatment with IVIG and MMF | |
ANA, antinuclear antibodies; AZA, azathioprine; BNB, baricitinib; CK, creatine kinase; CRP, C-reactive protein; DM, dermatomyositis; GI, gastrointestinal; αHMG-CoA-reductase, anti-3-hydroxy-3-methylglutaryl coenzyme A reductase antibody; IA, immunoadsorption; ICAP, international consensus on ANA patterns; IMNM, immune-mediated necrotizing myopathy; IVIG, intravenous immunoglobulin; LDH, lactate dehydrogenase; MMF, mycophenolate mofetil; MTX, methotrexate; RTX, rituximab; TAC, tacrolimus; αTIF-1γ, anti-transcriptional intermediary factor 1 gamma antibody; TOF, tofacitinib.