AC-9 - Clumpy Nucleolar

Irregular staining of the nucleoli and Cajal bodies with a peri-chromosomal staining at the metaphase plates. e.g. anti-fibrillarin.
Associação Antigênica
Doença Associada
Relevância Clínica (Primeiro Nível)
Found in patients with SSc.
If SSc is clinically suspected, it is recommended to perform a follow-up test for anti-U3RNP/fibrillarin antibodies; the antigen is included in disease specific immunoassays (i.e, SSc profile)
If confirmed as anti-U3RNP/fibrillarin reactivity by immunoassay, the clinical association is with diffuse SSc, increased incidence of pulmonary arterial hypertension, skeletal muscle disease, severe cardiac involvement, and gastrointestinal dysmotility.
Among SSc patients, anti-U3RNP/fibrillarin antibodies are most commonly found in African American and Latin American patients
Although some anti-U3RNP/fibrillarin immunoassays are commercially available, technical issues relating to the limited sensitivity of these immunoassays should be taken into consideration.
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Nandiwada SL, Peterson LK, Mayes MD, et al. Ethnic differences in autoantibody diversity and hierarchy: More clues from a US cohort of patients with systemic
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Okano Y, Steen VD, Medsger TA. Autoantibody to U3 nucleolar ribonucleoprotein (fibrillarin) in patients with systemic sclerosis. Arthritis Rheum 1992;35:95

Tormey VJ, Bunn CC, Denton CP, et al. Anti-fibrillarin antibodies in systemic sclerosis. Rheumatology 2001;40:1157–62.