Inflammatory Myopathy, IdiopathicPhase 2/3TIMEISMUSCLENCT05832034

Add-on Intravenous Immunoglobulins in Early Myositis

A Phase 2/3 study testing Immune Globulin Intravenous (Human) in people with Inflammatory Myopathy, Idiopathic and Dermatomyositis, run by Academisch Medisch Centrum - Universiteit van Amsterdam (AMC-UvA) at 1 site in Netherlands.

Active, not recruiting

Under way, not looking for participants. Everyone needed has enrolled. The study is still running.

Official record

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Trial summary

Who is it for?: Ages 18 years and older
Conditions the study is for: Inflammatory Myopathy, Idiopathic, Dermatomyositis, Antisynthetase Syndrome
What is being tested?: Immune Globulin Intravenous (Human)
Type of treatment: Injection or infusion
Could I receive a placebo?: Yes, some participants may receive a placebo
How many people: 44 participants
Where: 1 siteNetherlands
Study dates: 13 Sep 2021to Jul 2026
Phase: Phase 2/3
Are there results?: Not yet: the study is under way

Key requirements

Who can take part, as listed by the sponsor. Only the study team can confirm whether you are eligible, so take this list to your doctor.

Ages 18 years and older
All sexes
No healthy volunteers
Adult, Older adult

Who may be able to join

Adult patients (≥ 18 years) with IIM, according to diagnostic criteria:
Dermatomyositis
Polymyositis
Anti-synthetase syndrome
Immune mediated necrotizing myopathy
Overlap myositis

Show all 17Show fewer

Disease duration < 12 months
Minimal disability defined as at least 10% loss on Manual Muscle Testing (MMT) and abnormal scores on two other Core Set Measures (CSMs) of the international Myositis Assessment and Clinical Studies (IMACS) group (see 'Primary and secondary outcomes').
Patients are eligible for inclusion if they are treatment-naive, or if there is no clinical evident response (as carefully judged by the treating physician at a screening visit) to prior treatment with:
High dosed glucocorticoids, such as dexamethasone (e.g. 40 mg per day up to 4 days) or intravenous methylprednisolone (e.g. 1000 mg daily for three days), within 1 week prior to screening visit.
Daily dosed prednisone 1 mg/kg, or equivalent, used for up to 2 weeks prior to screening visit.
Treatment with low-dosed prednisone (max 20 mg daily) up to three months prior to screening visit.
Treatment with biologicals or other immunosuppressive or immunomodulatory treatment when meeting all of the following criteria:
Stable dose for the last 6 months
The biological or other immunosuppressive or immunomodulatory treatment has been approved for a non-muscular condition (e.g. hematological condition, eczema)
The biological or other immunosuppressive or immunomodulatory treatment is not known to induce inflammatory myopathy
Signed informed consent

Who cannot join

A potentially eligible patient who meets any of the following criteria will be excluded from participation in this study:
Severe muscle weakness (i.e. bedridden, severe dysphagia requiring a feeding tube, or respiratory muscle weakness (forced vital capacity below 50% of predicted in upright position)) necessitating more intensive treatment than standard glucocorticoids from the start.
Related to IVIg:
History of thrombotic episodes within 10 years prior to enrolment
Known allergic reactions or other severe reactions to any blood-derived product
Known Immunoglobulin A (IgA) deficiency and IgA serum antibodies

Show all 12Show fewer

Pregnancy or trying to conceive
Use of nephrotoxic medication
Conditions that are likely to interfere with:
Compliance (legally incompetent and/or incapacitated patients are excluded), or,
Evaluation of efficacy (e.g. due to severe pre-existing disability as a result of any other disease than myositis or due to language barrier)
Immunosuppressive medication or immunomodulatory treatment within the last 3 months (e.g. azathioprine, methotrexate, mycophenolate mofetil, tacrolimus, cyclophosphamide, cyclosporine, IVIg, biologicals, Janus kinase inhibitors, plasmapheresis).

Trial locations and contacts

1 site in 1 country.

Netherlands1 site

Department of Neurology, Amsterdam UMC, location AMC
Amsterdam, North Holland, 1105 AZ

Other trials in Netherlands

About this study

Official title: Treatment With add-on IVIg in Myositis Early In the diSease Course May be sUperior to Steroids Alone for Reaching CLinical improvemEnt

In patients with myositis early immunomodulation by intensive treatment ("hit-early/hit-hard" principle) may induce faster reduction of disease activity and prevent chronic disability. Intravenous immunoglobulin (IVIg) in addition to standard treatment with glucocorticoids may be beneficial for this purpose: add-on IVIg improved symptoms in steroid-resistant myositis, and first-line monotherapy IVIg led to a fast and clinically relevant response in a pilot study in nearly 50% of patients with myositis.

Read the detailed description

Considering the known effects of IVIg in idiopathic inflammatory myopathies (IIM), both as add-on therapy in refractory patients, as well as monotherapy in newly diagnosed IIM, we conducted a phase-2 double-blind placebo-controlled randomized trial to investigate the effect of add-on IVIg in patients with newly diagnosed IIM, who are treated with monotherapy prednisone.

Objective:

The primary aim of this trial is to examine whether the addition of early administered IVIg to standard therapy with prednisone in patients with newly diagnosed myositis leads to an improved clinical response after 12 weeks, compared to prednisone and placebo. Clinical response will be measured as the difference of the mean TIS after 12 weeks between intervention and control groups.

The secondary aims are to examine whether the intervention leads to a shorter time to improvement, and sustained positive effects on health-related quality of life, physical activity and fatigue, and a sustained reduction of muscle MRI abnormalities, as assessed up to 52 weeks.

Following a screening visit at the outpatient clinic, patients will be admitted to the neurology ward of the Amsterdam University Medical Center (AUMC) for the first infusion of study treatment. The remaining study medication will be administered at home, according to routine clinical practice for IVIg treatment in neuromuscular disorders in the Netherlands. A second and third study treatment will be administered at home after 4 and 8 weeks. At baseline and after 4, 8, 12, 26 and 52 weeks outcome assessments will be performed at the outpatient clinic. The outpatient study clinic visits at baseline and after 4, 12, 26 and 52 weeks will be combined with regular outpatient clinic visits.

The additional burden related to outcome assessments will consist of MRI muscle imaging after 12 weeks, blood sampling after 2, 4, 6, and 10 weeks and filling in questionnaires at baseline and after 4, 8, 12, 26 and 52 weeks. In addition, participants are asked to wear a watch three times in a period of 12 weeks and after 26 weeks.

Conditions: Inflammatory Myopathy, Idiopathic, Dermatomyositis, Antisynthetase Syndrome, Immune-Mediated Necrotizing Myopathy, Polymyositis, Myositis
Being tested: Immune Globulin Intravenous (Human), Placebo
Keywords: Myositis, Inflammatory myopathies, Intravenous immunoglobulins, Early symptomatic, Total Improvement Score
Medical subject headings: Myositis, Dermatomyositis, Antisynthetase syndrome, Polymyositis

What participants receive

What participants receive. In a randomised study each person is assigned to one group by chance.

Immune Globulin Intravenous (Human)Drug
Also called Nanogam
IVIg is 2 g/kg over 2 to 5 days at baseline, followed by 2 g/kg IV in 2 to 5 days after 4 and 8 weeks. The rate of infusion is controlled by means of an infusion pump. The first dosage (30 grams IVIg) will be administered on the neurology ward.
PlaceboDrug
Also called Sodium chloride or saline 0.9%
Placebo infusions, containing sodium chloride 0.9%, at baseline and after 4 and 8 weeks.

Study groups in NCT05832034
Study group	Role	What they receive
Add-on IVIg	Experimental	Patients in the intervention arm will be treated with Nanogam® in a dosage of 2 g/kg over 2-5 days, with a maximum of 80 g/day and a total of 180 gram at baseline and after 4 and 8 weeks. Nanogam® contains 100 mg/mL normal human immunoglobulin with a purity of at least 95% IgG and a maximum of 12 microgram/mL IgA, in a solution of water for injections and glucose. The first 30 grams are administered in hospital. Receives: Drug: Immune Globulin Intravenous (Human)
Placebo	Placebo comparator	Patients in the control arm will be treated with placebo infusions, containing sodium chloride 0.9%, at baseline and after 4 and 8 weeks. The first 30 grams are administered at the neurology ward, after 4 and 8 weeks infusions will be administered at home Receives: Drug: Placebo

What is being measured

The primary outcome is the main question the study is designed to answer.

Change in Total Improvement Score (TIS)
The primary outcome is the Total Improvement Score (TIS) of the myositis response criteria after 12 weeks, measured as the difference of the mean TIS after 12 weeks between intervention and control groups. Total Improvement Score (TIS) is based on 6 validated core set measures (CSMs), which each determine disease activity as defined by the International Myositis Assessment and Clinical Studies (IMACS) group. TIS ranges between 0 and 100 and corresponds to a degree of improvement; higher scores correspond to a greater degree of improvement.
Measured: Week 12

Secondary outcomes (21)

Total Improvement Score (IMACS).
Total Improvement Score (TIS) is based on 6 validated core set measures (CSMs), which each determine disease activity as defined by the International Myositis Assessment and Clinical Studies (IMACS) group. TIS ranges between 0 and 100 and corresponds to a degree of improvement; higher scores correspond to a greater degree of improvement.
Measured: TIS will be assessed at t = 0, and after 4, 8, 12, 26 and 52 weeks
Moderate improvement proportion
This is defined as proportion of patients in each treatment group that reaches moderate improvement, i.e. Total Improvement Score of ≥40. Total Improvement Score is based on 6 validated core set measures (CSMs), which each determine disease activity as defined by the International Myositis Assessment and Clinical Studies (IMACS) group.
Measured: Examined at week 12
Time to response (TIS>40 points)
This is defined as the time is taken to reach a Total Improvement Score > 40 points.Total Improvement Score is based on 6 validated core set measures (CSMs), which each determine disease activity as defined by the International Myositis Assessment and Clinical Studies (IMACS) group.
Measured: Will be examined at week 4, 8, 12, 26 and 52 weeks.
Core set measures (CSM) - physician global activity (PhGA)
1. physician global activity (PhGA): assessment of global disease activity on a 10 cm Visual Analogue Scale (VAS) by the treating physician. 0 = no disease activity, 10 most activity
On a total of 6 CSM
Measured: CSMs will be assessed at baseline, week 4, 8, 12, 26 and 52.
Core set measures (CSM) - patient global activity (PGA)
1. patient global activity (PGA): assessment of global disease activity on a 10 cm VAS by the patient. 0 = no disease activity, 10 most activity On a total of 6 CSM
Measured: CSMs will be assessed at baseline, week 4, 8, 12, 26 and 52.
Core set measures (CSM) - Manual Muscle Testing (MMT)
1. Manual Muscle Testing (MMT): sum score of 12 proximal+distal and 2 axial muscle groups. Score 0 - 260 (no muscle weakness). 0 = zero contractions in muscle, 10 = normal On a total of 6 CSM
Measured: CSMs will be assessed at baseline, week 4, 8, 12, 26 and 52.
Core set measures (CSM) - Health Assessment Questionnaire (HAQ)
1. Health Assessment Questionnaire (HAQ): average of a survey scoring 8 domains, from 0 (without any difficulty) to 3 (unable to do) On a total of 6 CSM
Measured: CSMs will be assessed at baseline, week 4, 8, 12, 26 and 52.
Core set measures (CSM) - Serum muscle enzyme activities
1. Serum muscle enzyme activities expressed as the most abnormal one in the upper limit of normal.
On a total of 6 CSM
Measured: CSMs will be assessed at baseline, week 4, 8, 12, 26 and 52.
Core set measures (CSM) - Extramuscular disease activity
1. Extramuscular disease activity on a 10 cm VAS based on the Myositis Disease Activity Assessment, measuring the degree of disease activity of extra-muscular organ systems. Score is based on a 0 - 4 scale, related to worsening or improvement.
On a total of 6 CSM
Measured: CSMs will be assessed at baseline, week 4, 8, 12, 26 and 52.
Patient-Reported Outcome Measures (PROMs) questionnaire - Fatigue
These PROMs relate to different aspects of quality of life, this questionnaire specified on fatigue. The three PROMs are offered as Short Forms: fixed set of 4-10 questions for one of each domain.
Each item is scored on a 5-point Likert scale, with higher scores indicating better functioning, and item category responses range from 1 to 5.
For example: 1= not at al, 2 = a little, 3 = moderately, 4 = mostly, 5= completely.
Measured: At baseline, and after 4, 8, 12, 26 and 52 weeks.
Patient-Reported Outcome Measures (PROMs) questionnaire - Pain interference
These PROMs relate to different aspects of quality of life, this questionnaire specified on pain interference (in life). The three PROMs are offered as Short Forms: fixed set of 4-10 questions for one of each domain.
Each item is scored on a 5-point Likert scale, with higher scores indicating better functioning, and item category responses range from 1 to 5.
For example: 1= not at al, 2 = a little, 3 = moderately, 4 = mostly, 5= completely.
Measured: At baseline, and after 4, 8, 12, 26 and 52 weeks.
Patient-Reported Outcome Measures (PROMs) questionnaire - Physical function
These PROMs relate to different aspects of quality of life, this questionnaire specified on physical function. The three PROMs are offered as Short Forms: fixed set of 4-10 questions for one of each domain.
Each item is scored on a 5-point Likert scale, with higher scores indicating better functioning, and item category responses range from 1 to 5.
For example: 1= not at al, 2 = a little, 3 = moderately, 4 = mostly, 5= completely.
Measured: At baseline, and after 4, 8, 12, 26 and 52 weeks.
Fatigue
The second most important domain according to patients with myositis and health-care providers (OMERACT study group). We will use the Checklist Individual Strength (CIS)-fatigue, a generic fatigue scale, which has been validated in neuromuscular disorders.
Measured: At baseline, and after 4, 8, 12, 26 and 52 weeks.
Health related quality of life (HR-QoL)
HR-QoL will be assessed with EuroQol Group Health Questionnaire (EQ5D). EQ5D is a widely used questionnaire for assessment of general health and has shown responsivity in our previous study on monotherapy IVIg in IIM
Measured: At baseline, and after 4, 8, 12, 26 and 52 weeks.
Physical activity
Accelerometry will be used to measure physical activity, patients will be offered a wrist-worn wearable (Actigraph GT9X32). For accelerometry we will calculate the mean number of steps and the mean number of flights of stairs per 24 hours, during the 5 most active days per week.
Measured: Two consecutive weeks, at baseline, week 4, week 8 and week 26.
Mean daily prednisone dosage
Start dosage 1 mg/kg (maximum 80 mg). A standard tapering scheme in the first months consists of 10 mg reduction of dosage every 4 weeks.
Measured: Calculated at week 4, 8, 12, 26 and 52.
MRI abnormalities
Indicative for edema (T2/STIR) and fatty infiltration (T1) on total body MRI. The MRI results will be used as a marker of inflammation and disease damage, respectively. Sum scores of semi-quantitatively rated muscle, fascial and subcutaneous edema and fatty infiltration will be calculated.
Measured: At baseline, and after 12 and 26 weeks.
IgG blood levels.
Immunoglobulin G (IgG) levels in serum samples will be measured by turbidimetry.
Measured: Obtained immediately before, immediately after and two weeks after the administration of study medication
Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI).
In the subgroup of patients with dermatomyositis, this validated tool will be used to characterize cutaneous dermatomyositis severity and detect improvement in disease activity.
Measured: At baseline, and after 4, 8, 12, 26 and 52 weeks.
Composite questionnaire on health care use and productivity loss.
This questionnaire is based on the Medical Consumption Questionnaire (iMCQ) and the Productivity Cost Questionnaire (iPCQ) and is currently being used in the OPTIC trial (add-on prednisone in chronic inflammatory demyelinating polyneuropathy (CIDP)).
Measured: At baseline and week 12
Interferon pathway markers
Serological biomarkers galectin-9, CXCL10, Siglec-1 are indicative of the interferon pathway and will be measured at week 0, 4, 8, 12, 26 and 52.
Measured: Examined at week 0, 4, 8, 12, 26 and 52.

Timeline

From the start of the study to its completion; the mark is today. Estimated dates are the sponsor’s plan.

13 Sep 2021Jul 2026

First posted27 Apr 2023
Study start13 Sep 2021
Primary completion25 Sep 2025
Study completionJul 2026 (estimated)

Resources

Official record on ClinicalTrials.govThe registry entry this page is built from, with its full change history.
Publications (7)Papers the sponsor has linked to this study.
Other trials for Inflammatory Myopathy, IdiopathicStudies of the same condition, open and under way.
More trials run by Academisch Medisch Centrum - Universiteit van Amsterdam (AMC-UvA)The sponsor's other registered studies.
What the trial phases meanHow a treatment moves from first tests in people to approval.

More details

For doctors and researchers: how the study is designed, who runs it, and its registry IDs.

Study design

Study type: Interventional
Phase: Phase 2/3
Primary purpose: Treatment
Allocation: Randomised
Intervention model: Parallel groups
Masking: Quadruple blind
Who is masked: Participant, Care provider, Investigator, Outcomes assessor
Enrolment: 44 participants (actual)

IDs, dates and publications

ClinicalTrials.gov ID: NCT05832034
Sponsor's protocol ID: NL74270.018.20
Acronym: TIMEISMUSCLE
Status last verified: Feb 2026
First submitted: 21 Feb 2022
Last updated: 27 Feb 2026
Results posted: No
Data monitoring committee: No
FDA-regulated drug: No
FDA-regulated device: No
Individual data sharing: No

Publications

Oddis CV, Aggarwal R. Treatment in myositis. Nat Rev Rheumatol. 2018 May;14(5):279-289. doi: 10.1038/nrrheum.2018.42. Epub 2018 Mar 29.
PubMed 29593343
Aggarwal R, Rider LG, Ruperto N, Bayat N, Erman B, Feldman BM, Oddis CV, Amato AA, Chinoy H, Cooper RG, Dastmalchi M, Fiorentino D, Isenberg D, Katz JD, Mammen A, de Visser M, Ytterberg SR, Lundberg IE, Chung L, Danko K, Garcia-De la Torre I, Song YW, Villa L, Rinaldi M, Rockette H, Lachenbruch PA, Miller FW, Vencovsky J; International Myositis Assessment and Clinical Studies Group and the Paediatric Rheumatology International Trials Organisation. 2016 American College of Rheumatology/European League Against Rheumatism criteria for minimal, moderate, and major clinical response in adult dermatomyositis and polymyositis: An International Myositis Assessment and Clinical Studies Group/Paediatric Rheumatology International Trials Organisation Collaborative Initiative. Ann Rheum Dis. 2017 May;76(5):792-801. doi: 10.1136/annrheumdis-2017-211400.
PubMed 28385805
Hoogendijk JE, Amato AA, Lecky BR, Choy EH, Lundberg IE, Rose MR, Vencovsky J, de Visser M, Hughes RA. 119th ENMC international workshop: trial design in adult idiopathic inflammatory myopathies, with the exception of inclusion body myositis, 10-12 October 2003, Naarden, The Netherlands. Neuromuscul Disord. 2004 May;14(5):337-45. doi: 10.1016/j.nmd.2004.02.006. No abstract available.
PubMed 15099594
Dalakas MC, Illa I, Dambrosia JM, Soueidan SA, Stein DP, Otero C, Dinsmore ST, McCrosky S. A controlled trial of high-dose intravenous immune globulin infusions as treatment for dermatomyositis. N Engl J Med. 1993 Dec 30;329(27):1993-2000. doi: 10.1056/NEJM199312303292704.
PubMed 8247075
Mecoli CA, Park JK, Alexanderson H, Regardt M, Needham M, de Groot I, Sarver C, Lundberg IE, Shea B, de Visser M, Song YW, Bingham CO 3rd, Christopher-Stine L. Perceptions of Patients, Caregivers, and Healthcare Providers of Idiopathic Inflammatory Myopathies: An International OMERACT Study. J Rheumatol. 2019 Jan;46(1):106-111. doi: 10.3899/jrheum.180353. Epub 2018 Sep 15.
PubMed 30219767
Malattia C, Damasio MB, Madeo A, Pistorio A, Providenti A, Pederzoli S, Viola S, Buoncompagni A, Mattiuz C, Beltramo A, Consolaro A, Ravelli A, Ruperto N, Picco P, Magnano GM, Martini A. Whole-body MRI in the assessment of disease activity in juvenile dermatomyositis. Ann Rheum Dis. 2014 Jun;73(6):1083-90. doi: 10.1136/annrheumdis-2012-202915. Epub 2013 May 1.
PubMed 23636654
Lim J, Eftimov F, Verhamme C, Brusse E, Hoogendijk JE, Saris CGJ, Raaphorst J, De Haan RJ, van Schaik IN, Aronica E, de Visser M, van der Kooi AJ. Intravenous immunoglobulins as first-line treatment in idiopathic inflammatory myopathies: a pilot study. Rheumatology (Oxford). 2021 Apr 6;60(4):1784-1792. doi: 10.1093/rheumatology/keaa459.
PubMed 33099648

Want to know more?

The sponsor has not listed a contact. The official record may have more details.

Talk to your doctor first and take this page with you. Only the study team can confirm whether you can join.

Questions to ask the study team

What is the study trying to find out?
What would I need to do, and how often would I visit?
Could I receive a placebo instead of the treatment?
What are the possible risks and benefits for me?
Will it cost me anything? Are travel or time paid for?
Can I keep seeing my own doctor during the study?
What happens when the study ends?

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Study at a glance

From the registry record, last updated by the sponsor on 27 Feb 2026. Recruitment status is as the sponsor reported it: confirm with the study team.

Official title: Treatment With add-on IVIg in Myositis Early In the diSease Course May be sUperior to Steroids Alone for Reaching CLinical improvemEnt
ClinicalTrials.gov ID: NCT05832034
Acronym / sponsor ID: TIMEISMUSCLE / NL74270.018.20
Conditions: Inflammatory Myopathy, Idiopathic, Dermatomyositis, Antisynthetase Syndrome, Immune-Mediated Necrotizing Myopathy, Polymyositis, Myositis
Keywords: Myositis, Inflammatory myopathies, Intravenous immunoglobulins, Early symptomatic, Total Improvement Score
Phase: Phase 2/3
Study type: Interventional
Recruitment status: Active, not recruiting
Enrolment: 44 participants (actual)
Lead sponsor: Academisch Medisch Centrum - Universiteit van Amsterdam (AMC-UvA)University, hospital or other
Collaborators: Princess Beatrix Muscle Foundation, Prothya Biosolutions
Study start: 13 Sep 2021 (actual)
Primary completion: 25 Sep 2025 (actual)
Study completion: Jul 2026 (estimated)
First posted: 27 Apr 2023 (actual)
Results: Not posted in the source record

Clinical summary

The research question as the sponsor registered it. This describes what the study plans to test, not what it found.

Detailed description

Objective:

Study design

Primary purpose: Treatment
Allocation: Randomised
Intervention model: Parallel groups
Masking: Quadruple blind (participant, care provider, investigator, outcomes assessor)
Number of arms: 2

Arm	Type	Receives
Add-on IVIg	Experimental	Drug: Immune Globulin Intravenous (Human)Patients in the intervention arm will be treated with Nanogam® in a dosage of 2 g/kg over 2-5 days, with a maximum of 80 g/day and a total of 180 gram at baseline and after 4 and 8 weeks. Nanogam® contains 100 mg/mL normal human immunoglobulin with a purity of at least 95% IgG and a maximum of 12 microgram/mL IgA, in a solution of water for injections and glucose. The first 30 grams are administered in hospital.
Placebo	Placebo comparator	Drug: PlaceboPatients in the control arm will be treated with placebo infusions, containing sodium chloride 0.9%, at baseline and after 4 and 8 weeks. The first 30 grams are administered at the neurology ward, after 4 and 8 weeks infusions will be administered at home

Who may qualify

Ages: 18 years and older
Sex: All
Healthy volunteers: Not accepted
Age groups: Adult, Older adult

Key inclusion criteria (17 listed)

Adult patients (≥ 18 years) with IIM, according to diagnostic criteria:
Disease duration < 12 months
Minimal disability defined as at least 10% loss on Manual Muscle Testing (MMT) and abnormal scores on two other Core Set Measures (CSMs) of the international Myositis Assessment and Clinical Studies (IMACS) group (see 'Primary and secondary outcomes').
Patients are eligible for inclusion if they are treatment-naive, or if there is no clinical evident response (as carefully judged by the treating physician at a screening visit) to prior treatment with:
High dosed glucocorticoids, such as dexamethasone (e.g. 40 mg per day up to 4 days) or intravenous methylprednisolone (e.g. 1000 mg daily for three days), within 1 week prior to screening visit.

Key exclusion criteria (12 listed)

A potentially eligible patient who meets any of the following criteria will be excluded from participation in this study:
Severe muscle weakness (i.e. bedridden, severe dysphagia requiring a feeding tube, or respiratory muscle weakness (forced vital capacity below 50% of predicted in upright position)) necessitating more intensive treatment than standard glucocorticoids from the start.
Related to IVIg:
Conditions that are likely to interfere with:
Immunosuppressive medication or immunomodulatory treatment within the last 3 months (e.g. azathioprine, methotrexate, mycophenolate mofetil, tacrolimus, cyclophosphamide, cyclosporine, IVIg, biologicals, Janus kinase inhibitors, plasmapheresis).

Eligibility is decided by the study team after screening; this overview does not establish that a patient qualifies. Full inclusion and exclusion criteria.

Interventions

Intervention	Type	Arms
Immune Globulin Intravenous (Human)Also: NanogamIVIg is 2 g/kg over 2 to 5 days at baseline, followed by 2 g/kg IV in 2 to 5 days after 4 and 8 weeks. The rate of infusion is controlled by means of an infusion pump. The first dosage (30 grams IVIg) will be administered on the neurology ward.	Drug	Add-on IVIg
PlaceboAlso: Sodium chloride or saline 0.9%Placebo infusions, containing sodium chloride 0.9%, at baseline and after 4 and 8 weeks.	Drug	Placebo

Primary outcome

Change in Total Improvement Score (TIS)
Time frame: Week 12
The primary outcome is the Total Improvement Score (TIS) of the myositis response criteria after 12 weeks, measured as the difference of the mean TIS after 12 weeks between intervention and control groups. Total Improvement Score (TIS) is based on 6 validated core set measures (CSMs), which each determine disease activity as defined by the International Myositis Assessment and Clinical Studies (IMACS) group. TIS ranges between 0 and 100 and corresponds to a degree of improvement; higher scores correspond to a greater degree of improvement.

21 secondary outcomes

Total Improvement Score (IMACS).
Time frame: TIS will be assessed at t = 0, and after 4, 8, 12, 26 and 52 weeks
Moderate improvement proportion
Time frame: Examined at week 12
Time to response (TIS>40 points)
Time frame: Will be examined at week 4, 8, 12, 26 and 52 weeks.
Core set measures (CSM) - physician global activity (PhGA)
Time frame: CSMs will be assessed at baseline, week 4, 8, 12, 26 and 52.
Core set measures (CSM) - patient global activity (PGA)
Time frame: CSMs will be assessed at baseline, week 4, 8, 12, 26 and 52.
Core set measures (CSM) - Manual Muscle Testing (MMT)
Time frame: CSMs will be assessed at baseline, week 4, 8, 12, 26 and 52.
Core set measures (CSM) - Health Assessment Questionnaire (HAQ)
Time frame: CSMs will be assessed at baseline, week 4, 8, 12, 26 and 52.
Core set measures (CSM) - Serum muscle enzyme activities
Time frame: CSMs will be assessed at baseline, week 4, 8, 12, 26 and 52.
Core set measures (CSM) - Extramuscular disease activity
Time frame: CSMs will be assessed at baseline, week 4, 8, 12, 26 and 52.
Patient-Reported Outcome Measures (PROMs) questionnaire - Fatigue
Time frame: At baseline, and after 4, 8, 12, 26 and 52 weeks.
Patient-Reported Outcome Measures (PROMs) questionnaire - Pain interference
Time frame: At baseline, and after 4, 8, 12, 26 and 52 weeks.
Patient-Reported Outcome Measures (PROMs) questionnaire - Physical function
Time frame: At baseline, and after 4, 8, 12, 26 and 52 weeks.
Fatigue
Time frame: At baseline, and after 4, 8, 12, 26 and 52 weeks.
Health related quality of life (HR-QoL)
Time frame: At baseline, and after 4, 8, 12, 26 and 52 weeks.
Physical activity
Time frame: Two consecutive weeks, at baseline, week 4, week 8 and week 26.
Mean daily prednisone dosage
Time frame: Calculated at week 4, 8, 12, 26 and 52.
MRI abnormalities
Time frame: At baseline, and after 12 and 26 weeks.
IgG blood levels.
Time frame: Obtained immediately before, immediately after and two weeks after the administration of study medication
Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI).
Time frame: At baseline, and after 4, 8, 12, 26 and 52 weeks.
Composite questionnaire on health care use and productivity loss.
Time frame: At baseline and week 12
Interferon pathway markers
Time frame: Examined at week 0, 4, 8, 12, 26 and 52.

Results and safety

This study is still under way. Results are not available in the source record yet.

Research links

Official record on ClinicalTrials.govLast updated by the sponsor 27 Feb 2026.
Background: PubMed 29593343Oddis CV, Aggarwal R. Treatment in myositis. Nat Rev Rheumatol. 2018 May;14(5):279-289. doi: 10.1038/nrrheum.2018.42. Epub 2018 Mar 29.
Background: PubMed 28385805Aggarwal R, Rider LG, Ruperto N, Bayat N, Erman B, Feldman BM, Oddis CV, Amato AA, Chinoy H, Cooper RG, Dastmalchi M, Fiorentino D, Isenberg D, Katz JD, Mammen A, de Visser M, Ytterberg SR, Lundberg IE, Chung L, Danko K, Garcia-De la Torre I, Song YW, Villa L, Rinaldi M, Rockette H, Lachenbruch PA, Miller FW, Vencovsky J; International Myositis Assessment and Clinical Studies Group and the Paediatric Rheumatology International Trials Organisation. 2016 American College of Rheumatology/European League Against Rheumatism criteria for minimal, moderate, and major clinical response in adult dermatomyositis and polymyositis: An International Myositis Assessment and Clinical Studies Group/Paediatric Rheumatology International Trials Organisation Collaborative Initiative. Ann Rheum Dis. 2017 May;76(5):792-801. doi: 10.1136/annrheumdis-2017-211400.
Background: PubMed 15099594Hoogendijk JE, Amato AA, Lecky BR, Choy EH, Lundberg IE, Rose MR, Vencovsky J, de Visser M, Hughes RA. 119th ENMC international workshop: trial design in adult idiopathic inflammatory myopathies, with the exception of inclusion body myositis, 10-12 October 2003, Naarden, The Netherlands. Neuromuscul Disord. 2004 May;14(5):337-45. doi: 10.1016/j.nmd.2004.02.006. No abstract available.
Background: PubMed 8247075Dalakas MC, Illa I, Dambrosia JM, Soueidan SA, Stein DP, Otero C, Dinsmore ST, McCrosky S. A controlled trial of high-dose intravenous immune globulin infusions as treatment for dermatomyositis. N Engl J Med. 1993 Dec 30;329(27):1993-2000. doi: 10.1056/NEJM199312303292704.
Background: PubMed 30219767Mecoli CA, Park JK, Alexanderson H, Regardt M, Needham M, de Groot I, Sarver C, Lundberg IE, Shea B, de Visser M, Song YW, Bingham CO 3rd, Christopher-Stine L. Perceptions of Patients, Caregivers, and Healthcare Providers of Idiopathic Inflammatory Myopathies: An International OMERACT Study. J Rheumatol. 2019 Jan;46(1):106-111. doi: 10.3899/jrheum.180353. Epub 2018 Sep 15.
Background: PubMed 23636654Malattia C, Damasio MB, Madeo A, Pistorio A, Providenti A, Pederzoli S, Viola S, Buoncompagni A, Mattiuz C, Beltramo A, Consolaro A, Ravelli A, Ruperto N, Picco P, Magnano GM, Martini A. Whole-body MRI in the assessment of disease activity in juvenile dermatomyositis. Ann Rheum Dis. 2014 Jun;73(6):1083-90. doi: 10.1136/annrheumdis-2012-202915. Epub 2013 May 1.
Background: PubMed 33099648Lim J, Eftimov F, Verhamme C, Brusse E, Hoogendijk JE, Saris CGJ, Raaphorst J, De Haan RJ, van Schaik IN, Aronica E, de Visser M, van der Kooi AJ. Intravenous immunoglobulins as first-line treatment in idiopathic inflammatory myopathies: a pilot study. Rheumatology (Oxford). 2021 Apr 6;60(4):1784-1792. doi: 10.1093/rheumatology/keaa459.
Individual participant dataSharing planned: No

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Referral and contact

AzTrials does not enrol participants or take referrals. Contact the study team or a site listed below; they decide who can take part. This study is not recruiting at the moment.

Raaphorst, MD, PhD principal investigator

Academisch Medisch Centrum - Universiteit van Amsterdam (AMC-UvA)

1 site in 1 country: see every site and its status.

Questions about this trial

What is this clinical trial studying?

The full registered description is in the About this study section and the official record.

Who may be eligible to take part?

The sponsor lists 17 inclusion and 12 exclusion criteria. Eligibility is decided by the study team after screening, so read the full criteria and contact a listed site.

Is this clinical trial currently recruiting?

The record says: under way, not looking for participants, as of the sponsor's last update on 27 Feb 2026. Status can change between updates, so confirm with the study contact before making plans.

Where is the study taking place?

The record lists 1 site in Netherlands. Whether a site is taking part right now should be confirmed with that site. See every site.

What are the possible benefits and risks?

They depend on the study and the treatment, and no study can promise a benefit. Some participants in this study may receive a placebo. The study team explains the known risks during informed consent, before anyone joins; the record itself does not list them.

What does this combined phase mean?

Phase 2 studies give the treatment to a larger group to see whether it has the intended effect and to keep checking safety.

Phase 3 studies compare the treatment with the current standard treatment or a placebo in a large group, to confirm how well it works and watch for side effects.

Not every study follows the same path, and some studies (observational studies, device studies) have no phase.

Are results available for this study?

This study is still under way. Results are not available in the source record yet.

How can a doctor refer a patient or contact the research team?

The record lists no contact. The official record names the sponsor, who can be asked directly. AzTrials does not enrol participants or take referrals.

What does the primary outcome measure?

Change in Total Improvement Score (TIS) (time frame: Week 12). It is what the study was designed to measure, not a finding.

Where can I read the original study information?

On the ClinicalTrials.gov record for NCT05832034, which also lists 7 linked publications.

Does a listing mean the treatment is approved or proven to work?

No. A listing means the study is registered. It does not show that the treatment is safe, works, or is approved by a regulator.

How often is this information updated?

AzTrials copies changed records from ClinicalTrials.gov every night and reloads the whole registry every week. The sponsor last updated this record on 27 Feb 2026.