*2.3.2. Anti-SS-B/La antibodies*

Anti-SS-B/La antibodies are less common and usually coexist with anti-SS-A antibodies, and their presence in other systemic diseases, especially in SLE (25% of patients with TRU), is associated with skin lesions (erythema, alopecia), inflammation of the serous membranes, leukopenia, symptoms of dryness and they often coexist with the presence of anti-SS-A antibodies (secondary SS) [28]. Rao et al. have noted that anti-SSB antibodies are important for the SLE diagnosis. They are associated with cheek erythema, alopecia, serositis, secondary Sjögren's syndrome, and hematological changes such as leukocytopenia, thrombocytopenia, and immunoglobulins elevation. They are often accompanied by the presence of anti-/Ro or anti-SSA/Ro52 antibodies [34]. These autoantibodies may also be found before the SLEspecific antibodies can be detected [35].

anti-SS-A antibodies, anti-SS-B, increased ESR and leukopenia were presented in their work by Witte et al. [39]. The presence of RF in patients with pSS, as well as in other autoimmune diseases, and in acute infections, indicates the formation of a large number of other antibodies and the formation of antigen complexes with antibodies. The frequency of RF in various CTD

**Autoimmune connective tissue disease RF frequency%**

Primary Sjögren's Syndrome and Autoantibodies http://dx.doi.org/10.5772/intechopen.75011 125

Primary Sjögren's syndrome 75–90 Rheumatoid arthritis 70–90 Mixed connective tissue disease 50–60 Systemic sclerosis 20–30 Systemic lupus erythematosus 15–20 Dermato/polymyositis 20 Vasculitis (GPA and MPA) 5–20 Other CTD\* as psoriatic arthritis, juvenile idiopathic arthritis, and reactive arthritis 5 and <5

**Table 3.** The frequency of RF in various connective tissue diseases [39].

Anti-centromere antibodies (ACA) are directed to six antigens associated with centromere (composed of a complex of kinetochore proteins). Currently identified anti-centromere antibodies (CENP) have been assigned designations with letters from A to F. The most common are CENP-A, B and C. CENP-B is also the most frequently occurring anti-centromere antibody in patients with pSS. The incidence of ACA antibody described in the literature ranges from 3.7 to 4% [40, 41]. This antibody, with a mass of 80 kDa, is a DNA-binding protein involved in the heterochromatin folding. Anti-centromere antibodies (A, B, C) occur mainly in limited systemic sclerosis (LSSc) and are associated with the prevalence of telangiectasia, higher severity of Raynaud's symptom, lung involvement such as interstitial lung disease [ILD] and fibrosis [42]. Their relationship between the presence of ACA antibodies and the involvement of endocrine glands has been demonstrated; in the ACA+ group, anti-SS-A and

The association of ACA antibodies with non-Hodgkin's lymphomas (CENP-F) including MALT lymphomas was also described [43], as well as the case reports of CENP-B presence in

Interestingly, it has also been demonstrated that the presence of anti-CENP-B antibodies is

was presented in **Table 3** [39].

**4. Other autoantibodies**

**4.1. Anti-centromere autoantibodies**

anti-SS-B autoantibodies were less frequent [42].

associated with prolonged survival in a breast cancer [45].

small-cell lung cancer [44].
