RP11

check icon Optimal Result: 0 - 11 SI.

RNA polymerase III antibodies target RNA polymerase epitopes 11 and 155 and are thus also known as anti-RP11 and anti-RP155.

These antibodies are found in 7% to 41% of patients with SSc and occur most often in dcSSc.

They are diagnostic for SSc, as they are rarely found in other autoimmune diseases, and are included in the 2013 ACR-EULAR classification criteria.

The presence of RNA polymerase III antibodies is associated with progressive skin thickening, gastric antral vascular ectasia (GAVE), and renal crisis.

In addition, these antibodies are associated with onset of cancer within a 2-year timeframe before or after onset of SSc skin changes. Historically, RNA polymerase III antibodies indicated a poor prognosis, but mortality rates improved after the introduction of ACE inhibitors to treat renal crisis; the prognosis for patients with RNA polymerase III antibodies is now better than for those with Scl-70 or U3-RNP antibodies.

Systemic Sclerosis Classification Criteria:

Classify as having systemic sclerosis if the sum of points for criteria below is ≥9.

Criterion

Points

Skin thickened on fingers of both hands, extending proximal to the metacarpophalangeal joints

9

Skin on fingers thickened (only count highest score)

   Puffy fingers

   Sclerodactylya

 

2

4

Lesions on fingertips (only count highest score)

   Ulcers on tip of digits

   Pitting scars on fingertips

 

2

3

Telangiectasia

2

Abnormal nailfold capillaries

2

Pulmonary arterial hypertension and/or interstitial lung disease (max score is 2)

   Pulmonary arterial hypertension

   Interstitial lung disease

 

2

2

Raynaud phenomenon

3

Presence of 1 or more of the following:

   Centromere antibody

   Scl-70 antibody

   RNA polymerase III antibody

3

Clinical Characteristics Associated With Autoantibodies Used in the Diagnosis and Classification of Systemic Sclerosisa:

Antibody

Prevalence in SSc, %

Presence in other autoimmune diseases

SSc type most likely

Clinical associations

Relative SSc prognosis

ANA

85-95

Common

Does not help differentiate type

Most common in rheumatic diseases

Varies

Centromere A or B

20-40 b

Uncommon

lcSSc

PAH, calcinosis, digital ischemia, intestinal involvement

Better

Ku

1.5-5

Common

Overlap syndromes or lcSSc

Muscle and joint involvement

Unclear

PM/Scl-75 or PM/Scl-100

2-11

Common

SSc-myositis overlap syndrome or lcSSc

Muscle and joint involvement, calcinosis; GI and lung involvement for those with PM/Scl-75

Better

RNA Polymerase III

7-41

Rare

dcSSc

Renal crisis, progressive skin thickening, GAVE, malignancy

Poorer

Scl-70

9.4-71 b

Rare

dcSSc

Severe pulmonary fibrosis, cardiac involvement, renal crisis, digital ulcers

Poorer

Th/To

2-5

Rare

lcSSc

Pulmonary involvement

Poorer

U1-snRNP

2-14

Common (90% in patients with MCTD)

lcSSc

PAH, arthritis, esophageal dysfunction

Better

U3-RNP (Fibrillarin)

4-10 b

Rare

dcSSc

Multiorgan involvement, PAH

Poorer

ANA, antinuclear antibodies; dcSSc, diffuse cutaneous systemic sclerosis; GAVE, gastric antral vascular ectasia; GI, gastrointestinal; lcSSc, limited cutaneous systemic sclerosis; MCTD, mixed connective tissue disease; PAH, pulmonary arterial hypertension; SSc, systemic sclerosis.
a Data presented in this table have been compiled from the cited references. It is important to note that autoantibody frequency in SSc as well as other autoimmune diseases varies with sex, ethnicity, and population studied.
b In the United States, prevalence has been shown to vary across ethnicities.

References:

van den Hoogen F, Khanna D, Fransen J, et al. 2013 classification criteria for systemic sclerosis: an American College of Rheumatology/European League against Rheumatism collaborative initiative. Arthritis Rheum. 2013;65(11):2737-2747. doi:10.1002/art.38098

Stochmal A, Czuwara J, Trojanowska M, et al. Antinuclear antibodies in systemic sclerosis: an update. Clin Rev Allergy Immunol. 2020;58(1):40-51. doi:10.1007/s12016-018-8718-8

Hamaguchi Y, Takehara K. Anti-nuclear autoantibodies in systemic sclerosis: news and perspectives. J Scleroderma Relat Disord. 2018;3(3):201-213. doi:10.1177/2397198318783930

Shah AA, Hummers LK, Casciola-Rosen L, et al. Examination of autoantibody status and clinical features associated with cancer risk and cancer-associated scleroderma. Arthritis Rheumatol. 2015;67(4):1053-1061. doi:10.1002/art.39022

Sobanski V, Dauchet L, Lefèvre G, et al. Prevalence of anti-RNA polymerase III antibodies in systemic sclerosis: new data from a French cohort and a systematic review and meta-analysis. Arthritis Rheumatol. 2014;66(2):407-417. doi:10.1002/art.38219

What does it mean if your RP11 result is too high?

RNA polymerase III antibodies target RNA polymerase epitopes 11 and 155 and are thus also known as anti-RP11 and anti-RP155.

These antibodies are found in 7% to 41% of patients with SSc and occur most often in dcSSc.

They are diagnostic for SSc, as they are rarely found in other autoimmune diseases, and are included in the 2013 ACR-EULAR classification criteria.

The presence of RNA polymerase III antibodies is associated with progressive skin thickening, gastric antral vascular ectasia (GAVE), and renal crisis.

In addition, these antibodies are associated with onset of cancer within a 2-year timeframe before or after onset of SSc skin changes. Historically, RNA polymerase III antibodies indicated a poor prognosis, but mortality rates improved after the introduction of ACE inhibitors to treat renal crisis; the prognosis for patients with RNA polymerase III antibodies is now better than for those with Scl-70 or U3-RNP antibodies.

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