ICAP
AC-24 - Centrosome
Previous Nomenclature Centrioles
Description Distinct centrioles (1-2/cell) in cytoplasm and at the poles of mitotic spindle.
Antigen Association pericentrin, ninein, Cep250, Cep110
Clinical Relevance
First level information
About Clinical Relevance & List of Abbreviations

The AC-24 pattern has low positive predictive value for any disease

Within the spectrum of the SARD, the AC-24 pattern is found in patients with Raynaud’s phenomenon, localized scleroderma, SSc, SLE and RA, either alone or in combination with other SSc-associated antibodies (102–105)

Antigens recognized include α-enolase, γ-enolase, ninein, Cep-250, Mob1, PCM-1/2, pericentrin; specific immunoassays for these autoantibodies are currently not commercially available (104, 106–108)

First level information references
102.

Takahashi T, Asano Y, Hirakawa M, et al. Linear scleroderma with prominent multiple lymphadenopathy followed by the development of polymyositis: a case report and review of published work. J Dermatol 2016;43:1224-7.

103.

Howng SL, Chou AK, Lin CC, et al. Autoimmunity against hNinein, a human centrosomal protein, in patients with rheumatoid arthritis and systemic lupus erythematosus. Mol Med Rep 2011;4:825-30.

104.

Hamaguchi Y, Matsushita T, Hasegawa M, et al. High incidence of pulmonary arterial hypertension in systemic sclerosis patients with anti-centriole autoantibodies. Mod Rheumatol 2015;25:798-801.

105.

Gavanescu I, Vazquez-Abad D, McCauley J, et al. Centrosome proteins: a major class of autoantigens in scleroderma. J Clin Immunol 1999;19:166-71.

106.

Fritzler MJ, Zhang M, Stinton LM, et al. Spectrum of centrosome autoantibodies in childhood varicella and post-varicella acute cerebellar ataxia. BMC Pediatr 2003;3.

107.

Rattner JB, Martin L, Waisman DM, et al. Autoantibodies to the centrosome (centriole) react with determinants present in the glycolytic enzyme enolase. J Immunol 1991;146:2341-4.

108.

Mack GJ, Rees J, Sandblom O, et al. Autoantibodies to a group of centrosomal proteins in human autoimmune sera reactive with the centrosome. Arthritis Rheum 1998;41:551-8.

Second level information

Possible association with infections; described in children with Mycoplasma pneumoniae infection (1)

Possible association with malignancies; autoantibodies reacting with antigens in centrosomes are frequently found in sera of patients with breast cancer (2, 3)

Autoantibodies to α-enolase, γ-enolase, Mob1, PCM-1/2, and pericentrin have been described in children with cerebellar ataxia after varicella infection; one patient with hyperthyroidism and vague muscle pain and one patient with Raynaud’s phenomenon, both without evidence of a SARD, had antibodies to α-enolase and γ-enolase (4.5)

With respect to autoantibodies to ninein and Cep-250, there is no apparent correlation between serum autoantibody reactivity and the clinical diagnosis; reported in RA and SLE (6, 7)

 

Notes: Most reports describe autoantibodies directly binding to specific antigens (i.e., antigen-specific immunoassays) and do not actually show correlations with the AC-24 pattern as such; specific immunoassays for these autoantibodies are currently not commercially available.

Second level information references
1.

Cimolai N, Mah D, Roland E. Anticentriolar autoantibodies in children with central nervous system manifestations of Mycoplasma pneumonia infection. J Neurol Neurosurg Psychiatry 1994;57:638-9.

2.

Madrid FF, Maroun MC, Olivero OA, et al. Autoantibodies in breast cancer sera are not epiphenomena and may participate in carcinogenesis. BMC Cancer 2015;15:407.

3.

Maroun MC, Olivero O, Lipovich L, et al. Anti-centrosome antibodies in breast cancer are the expression of autoimmunity. Immunol Res 2014;60:339-47.

4.

Fritzler MJ, Zhang M, Stinton LM, et al. Spectrum of centrosome autoantibodies in childhood varicella and post-varicella acute cerebellar ataxia. BMC Pediatr 2003;3:11.

5.

Rattner JB, Martin L, Waisman DM, et al. Autoantibodies to the centrosome (centriole) react with determinants present in the glycolytic enzyme enolase. J Immunol 1991;146:2341-4.

6.

Mack GJ, Rees J, Sandblom O, et al. Autoantibodies to a group of centrosomal proteins in human autoimmune sera reactive with the centrosome. Arthritis Rheum 1998;41:551-8.

7.

Howng SL, Chou AK, Lin CC, et al. Autoimmunity against hNinein, a human centrosomal protein, in patients with rheumatoid arthritis and systemic lupus erythematosus. Mol Med Rep 2011;4:825-30.

FAQ
How to deal with just a “nuclear speckled” IFA report?
In my practice I have followed patients with ANA findings, with a nuclear speckled pattern (without specifying whether fine/dense/coarse), in patients with very heterogeneous phenotypes, some with a clinical picture that suggests further investigation of systemic autoimmune disease (one patient with proximal muscle weakness and skin thickening) and others who represent only non-specific findings. In such situations, as a precaution, I request more specific autoantibodies. However, this pattern (nuclear speckled pattern) is not described by the "ICAP" and I am in doubt about which antigenic association it represents, even to guide which autoantibody may be present and which ones to look after. How to interpret this pattern? Does the lab describe it when it is not possible to "refine" such a conclusion? Could this be associated with deficiency in the methodology, sample, interpretation?
 
 
Online since 19 May 2015