Patient case (clinical description): A 50-year-old woman comes to the clinic complaining of difficulty swallowing both liquids and solid foods, as well as occasional cough while eating.  She also has difficulty lifting her arms above her head and getting up from a chair. The weakness seems to get worse gradually. The patient has no prior medical problems and takes no medications.  Vital signs are normal.  Physical examination shows an erythematous rash on the upper eyelids. There are some red papules over joints of her hands. The rest of the physical examination is unremarkable. Antinuclear antibodies, anti-Jo-1 antibodies and anti-MDA5 antibody are positive.  Muscle biopsy shows perifascicular inflammation and atrophy of the fascicle and surrounding blood vessels.

Patient's own account: I had to go to the clinic because of difficulty swallowing all kinds of food, liquid and solid. I also sometimes cough when I eat my dinner. At only 50 year old, I had some difficulties lifting up my arms and getting up from a chair! And it's only getting worse! I never had any medical issues in the past and I'm not under any medication, I just don't get it! My vitals were normal. The physical exam showed a red rash on my upper eyelids. I also have red papules on my hands' joints. After a blood test, they told me that my antinuclear antibodies, anti-Jo-1 antibodies, and anti-MDA5 antibodies were positive.  I also did a muscle biopsy and it showed that I had an atrophy of my blood vessels around my muscle fibers and that I have an inflammation of these.

Clinical trial: Abatacept Treatment in Polymyositis and Dermatomyositis

Eligibility criteria:
Inclusion Criteria:

          1. Patients with definite or probable polymyositis or dermatomyositis diagnosed according
             to the diagnostic criteria by Bohan and Peter (30,31).

          2. For polymyositis a muscle biopsy is required that confirmed this disease (performed at
             any time before the start of the study) and to exclude other conditions unless a
             patient is positive for myositis specific or myositis associated autoantibodies.

          3. Polymyositis will be included after a judicial process by the three PIs.

          4. Inflammatory active disease based on persisting or worsening muscle weakness, MMT <
             150 or low endurance, FI -2 < 20% of upper value together with at least one other sign
             of active disease: elevated serum levels of muscle enzymes (CK, LD, ASAT, ALAT, above
             upper limit and being explained by muscle involvement and not eg liver disease),
             inflammation in recent muscle biopsy (< 3 months) or on MRI scans or active
             extramuscular disease: skin rash, arthritis or interstitial lung disease (ILD) (as
             suggested by chest X-ray, high resolution computerized tomography (HRCT), or pulmonary
             function test).

          5. Persisting disease activity after a minimum of 3 months treatment with prednisolone or
             equal drug. The treatment with prednisolone (or equal) should include the dose of at
             least 0.5 mg/kg/day for at least 1 month in the history and should be stable 1 month
             prior the baseline visit. Maximum dose of glucocorticoids should not exceed the
             equivalent of Prednisone 30 mg per day at the time of randomisation.

          6. Combination with at least one other immunosuppressive drug, which includes
             methotrexate (minimum dose 15 mg/week) or azathioprine (minimum dose 100 mg/day) for
             at least 3 months. The dose of methotrexate or azathioprine should be stable for at
             least 1 month before the first administration of abatacept.

          7. If methotrexate or azathioprine has had to be stopped or the dose decreased due to
             documented intolerance, lower dose or absence of these drugs is accepted provided this
             situation is stable for 1 month before the baseline.

          8. Age between 18 - 80 years.

          9. Signed informed consent.

             -

        Exclusion Criteria:

          1. Patients with other types of inflammatory myopathies including

               -  Drug induced myositis.

               -  Inclusion body myositis

               -  Malignancy associated myositis.

          2. Women who are pregnant or breastfeeding.

          3. Women with a positive pregnancy test on enrolment or prior to start of study drug
             administration.

          4. Women of Child Bearing age who are unwilling or unable to use an acceptable method to
             avoid pregnancy for the entire study period and for 10 weeks after the last infusion
             of study medication.

          5. Current symptoms of severe, progressive, or uncontrolled renal, hepatic,
             hematological, gastrointestinal, pulmonary, cardiac, neurological, ophthalmologic or
             cerebral disease with the exception of those symptoms that are a manifestation of
             polymyositis or dermatomyositis. Concomitant medical conditions that in the opinion of
             the investigator might place the subject at unacceptable risk for participation in
             this study.

          6. Subjects with a history of cancer within the last five years (other than non-melanoma
             skin cell cancers cured by local resection). Existing non-melanoma skin cell cancers
             must be removed prior to dosing. Patients with dermatomyositis need to be screened for
             malignancies according to routine procedures.

          7. Subjects who have a history of clinically significant drug or alcohol abuse. Subjects
             currently taking methotrexate who admit to consumption of more than an average of 1
             alcoholic drink per day.

          8. Subjects with any serious bacterial infection (such as pneumonia, other renal
             infection and sinusitis), unless treated and resolved with antibiotics or chronic
             bacterial infection (such as pyelonephritis and chest infection with bronchiectasis)
             in the previous 3 months.

          9. Subjects with active tuberculosis requiring treatment within the previous 3 years.
             Subjects with a positive PPD at screening will not be eligible for the study unless
             they completed treatment for latent TB and have a negative chest x-ray at enrolment. A
             PPD response that is equal to or greater than 10 mm should be considered a positive
             test, although more conservative criteria may be applied as determined by the clinical
             circumstance and investigator according to published guidelines and/or local standards
             endorsed by the medical society. Quantiferon assay may replace PPD testing and
             patients are excluded from the study if the test is positive. Quantiferon positive
             patients who completed treatment for latent tuberculosis according to the local
             guidelines may be considered for enrolment.

         10. Subjects with herpes zoster that resolved less than 2 months prior to enrolment.

         11. Subjects with evidence (as assessed by the Investigator) of active or latent bacterial
             or viral infections at the time of potential enrolment, including subjects with
             evidence of Human Immunodeficiency Virus (HIV) infection.

         12. Significant toxicities associated with concomitant or previous immunosuppressive
             Therapy and anti-TNF therapy that would preclude subjects from participating and
             completing the study

         13. Patients with clinically apparent immunodeficiency syndrome, (IgA deficiency alone is
             not an exclusion criterion)

         14. Subjects with any of the following laboratory values:

               -  Hgb < 8.5 g/dL.

               -  WBC < 3,000/mm3 (3 x 109/L).

               -  Platelets < 100,000/mm3 (100 x 109/L).

               -  Serum creatinine > 2 times upper limit of normal.

               -  Any other laboratory test results that, in the opinion of the investigator, might
                  place the subject at unacceptable risk for participation in this study.

         15. Subjects previously treated with rituximab: B cell levels are less than lower limit of
             normal as measured by Fluorescent Activated Cell Sorting (FACS) analysis.

         16. For all patients who have received prior rituximab, a normal CD19 B cell count must be
             documented at the time of screening for this study.

         17. Patients with Large granular lymphocyte (LGL) syndrome (to minimize the risk of
             including patients with high frequency of CD28 null T cells) that may not benefit from
             treatment.

             -
No condition on gender to be admitted to the trial.
No healthy subjects accepted to join the trial.
Subject must be at least 18 Years old.
Subject must be at most 80 Years

Eligibility (NLI label): Entailment