creatine kinase
Anti-Jo-1 antibody testing
Electromyography
Muscle biopsy
Pulmonary function testing
Lung biopsy
Imaging such as High Resolution computed tomography In certain situations, testing of other antibodies, specific imaging (MRI, thoracic high resolution computed tomography), and swallowing evaluation may be needed. Treatment
Unfortunately, treatment for the anti-synthetase syndrome is limited, and usually involves immunosuppressive drugs such as glucocorticoids. For patients with pulmonary involvement, the most serious complication of this syndrome is pulmonary fibrosis and subsequent pulmonary hypertension.Additional treatment with azathioprine and/or methotrexate may be required in advanced cases. Prognosis
Prognosis is largely determined by the extent of pulmonary damage. References


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Triethylenetetramine (TETA and trien), also known as trientine (INN) when used medically, is an organic compound with the formula [CH2NHCH2CH2NH2]2. The pure freebase is a colorless oily liquid, but, like many amines, older samples assume a yellowish color due to impurities resulting from air-oxidation. It is soluble in polar solvents. The branched isomer tris(2-aminoethyl)amine and piperazine derivatives may also be present in commercial samples of TETA. The hydrochloride salts are used medically as a treatment for copper toxicity. Uses
The reactivity and uses of TETA are similar to those for the related polyamines ethylenediamine and diethylenetriamine. It is primarily used as a crosslinker ("hardener") in epoxy curing. Medical uses
The hydrochloride salt of TETA, referred to as trientine hydrochloride, is a chelating agent that is used to bind and remove copper in the body to treat Wilsons disease, particularly in those who are intolerant to penicillamine. Some recommend trientine as first-line treatment, but experience with penicillamine is more extensive.Trientine hydrochloride (brand name Syprine) was approved for medical use in the United States in November 1985.Trientine tetrahydrochloride (brand name Cuprior) was approved for medical use in the European Union in September 2017. It is indicated for the treatment of Wilsons disease in adults, adolescents and children five years of age or older who are intolerant to D-penicillamine therapy.Trientine dihydrochloride (brand name Cufence) was approved for medical use in the European Union in July 2019.