• It is characterized by the presence of high titer of anti-Ul-RNP antibodies.
• While some patients may differentiate into specific diseases such as SLE, scleroderma, some patients remain with MCDH.
• The onset of the disease is typically Raynaud's phenomenon, puffy fingers and myalgia.
• Other findings;
o Sclerodactyly, calcinosis, telangiectasia
o Malar rash, photosensitivity
o Heliotrope rash, Gottron papules
o Arthralgia
o Pulmonary fibrosis, isolated pulmonary arterial hypertension
o Esophageal dysmotility
o Sjogren's syndrome
o Kidney involvement (membranous glomerulonephritis)