Prospective study of a systemic sclerosis/dermatomyositis overlap patient presenting with anti-Ku and anti-Ki antibodies

Abstract

A 60-year-old woman visited the Keigu Clinic in January 1998 complaining of morning stiffness and flexion contracture of the distal interphalangeal joint. Blood tests showed the presence of antinuclear antibody at a 1 : 40 dilution with speckled staining. She was suspected of having Heberden's node. Nine months later, she developed Raynaud's phenomenon, sclerodactyly, and Gottron's sign, and was diagnosed with systemic sclerosis/dermatomyositis (SSc/DM) overlap. Blood tests revealed the presence of antinuclear antibody at a 1 : 5120 dilution, along with high titer of anti-Ku and anti-Ki antibodies. Subsequently, the patient developed interstitial pneumonia in January 2000. It is thought that the appearance of antinuclear antibody and development of other immunological events played an important role in determining this patient's limited SSc/DM overlap.

Comprehensive autoantibody profiling in systemic autoimmunity by a highly-sensitive multiplex protein array

“…In addition to anti-topoisomerase I antibody and anti-SS-B antibody detected by conventional ELISA, A-Cube revealed anti-RNA polymerase II antibody, anti-U1RNP_C antibody, anti-Ku antibody, and anti-Ki antibody. Among them, anti-U1RNP_C antibody, anti-Ku antibody, and anti-Ki antibody supported the overlap of myositis with SSc (25)(26)(27)(28). Following the systemic evaluation, myositis was successfully treated with oral prednisolone 40 mg/day.…”

Significance of Autoantibodies to Ki/SL as Biomarkers for Systemic Lupus Erythematosus and Sicca Syndrome

“…Several case reports have been published on patients exhibiting anti-Ki/SL antibodies [ 13 , 17 , 18 , 19 , 20 ], including a patient with fatal CTD overlap syndrome [ 13 ], a patient with SSc/dermatomyositis (DM) overlap syndrome, an individual with anti-centromere positive pulmonary-renal syndrome [ 18 ], a case with SLE with epileptic seizures and chorea during prednisolone treatment [ 16 ], an individual with SSc with interstitial pneumonia and various autoantibodies (improvement by intravenous cyclophosphamide therapy) [ 20 ] ( Table 1 ). In addition to the studies measuring anti-Ki/SL antibodies during a single timepoint (mostly at diagnosis), one case report also provided longitudinal analysis.…”