AC-9 - Clumpy Nucleolar
Descrição
Irregular staining of the nucleoli and Cajal bodies with a peri-chromosomal staining at the metaphase plates. e.g. anti-fibrillarin.
Associação Antigênica
U3-snoRNP/fibrillarin
Doença Associada
SSc
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.
Referências
Arnett FC, Reveille JD, Goldstein R, et al. Autoantibodies to fibrillarin in systemicsclerosis (scleroderma). An immunogenetic, serologic, and clinical analysis. Arthritis
Rheum 1996;39:1151–60.
Johnson SR, Fransen J, Khanna D, et al. Validation of potential classification criteria for systemic sclerosis. Arthritis Care Res 2012;64:358–67.
Mehra S, Walker J, Patterson K, et al. Autoantibodies in systemic sclerosis. Autoimmun Rev 2013;12:340–54.
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
sclerosis. J Rheumatol 2016;43:1816–24.
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.