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Brief Report ARTHRITIS & RHEUMATISM Vol. 64, No. 12, December 2012, pp 4066–4071 DOI 10.1002/art.34679 © 2012, American College of Rheumatology BRIEF REPORT Adrenal Autoimmunity in Primary Sjo¨gren’s Syndrome Clio P. Mavragani,1 Marian Schini,1 Fotini Gravani,2 Gregorios Kaltsas,1 and Haralampos M. Moutsopoulos1 Objective. To evaluate the prevalence of anti- Conclusion. Adrenal autoimmunity occurs in al- bodies to 21-hydroxylase (anti-21[OH]), a marker of most 20% of patients with primary SS in association autoimmune adrenal disease, in a cohort of patients with markers of B cell activation. Although the presence with primary Sjo¨gren’s syndrome (SS) and to investi- of adrenal autoantibodies was not associated with adre- gate whether the presence of anti-21(OH) correlates nal insufficiency in the present study, there was a with clinical, serologic, and salivary gland features of blunted adrenal response, suggesting the need for fur- the disease. ther followup and monitoring of adrenal function in Methods. Sera from 63 consecutive patients with patients with primary SS who are positive for the primary SS, 32 patients with autoimmune thyroid dis- autoantibodies. ease (AITD), and 20 healthy controls were obtained and anti-21(OH) levels were determined by radioimmuno- Primary Sjo¨gren’s syndrome (SS) is a chronic assay. Clinical, serologic, and histopathologic features autoimmune disease characterized by lymphocytic in- were recorded, and a short Synacthen test was used to filtration of salivary and lachrymal glands, clinically assess adrenal function reserve. Seven available minor manifesting as oral and ocular dryness, respectively. salivary gland (MSG) tissue specimens from patients B cell activation has long been considered a disease in the primary SS cohort were also assessed for hallmark associated with hypergammaglobulinemia, ␣ ␣ interferon- (IFN ), BAFF, and interleukin-21 (IL-21) cryoglobulinemia, and autoantibody production (1). cytokine transcripts, which are all implicated in B cell Furthermore, it has been proposed that type I interferon activation. (IFN)/BAFF axis activation (2–5) and, recently, Results. Anti-21(OH) positivity was detected in interleukin-21 (IL-21) (6) are significant pathogenetic 17.5% and 28.1% of primary SS and AITD patients, contributors. The autoantibodies most commonly found respectively, and in none of the healthy controls. While in sera from patients are against non–organ-specific no evidence of adrenal insufficiency was detected in any antigens, such as Ro/SSA–La/SSB ribonucleoprotein of the patients studied, a blunted rate of increase in complexes. In addition, it has been previously reported cortisol levels was observed in patients with detectable that other, organ-specific autoantibodies, such as anti- serum autoantibodies against 21(OH), compared to bodies against thyroid and mitochondrial antigens, fre- their anti-21(OH)–negative counterparts. A strong cor- quently occur in SS overlapping with autoimmune thy- relation between the serum titer of anti-21(OH) anti- roid disease (AITD) and primary biliary cirrhosis, bodies and expression of IFN␣, BAFF, and IL-21 mes- respectively (1). senger RNA in MSG tissues was also detected. Abnormalities of the hypothalamic–pituitary– adrenal (HPA) axis in patients with primary SS, which Supported by an unrestricted educational grant from Pfizer manifest as blunted cortisol responses to corticotropin- (to Dr. Moutsopoulos). 1Clio P. Mavragani, MD, Marian Schini, MD, Gregorios releasing hormone stimulation, have been previously Kaltsas, MD, FRCP, Haralampos M. Moutsopoulos, MD, FACP, reported and were attributed to either blunted adreno- FRCP: University of Athens, Athens, Greece; 2Fotini Gravani, MD: corticotropic hormone (ACTH) release from the pitu- General Hospital of Athens “G. Gennimatas,” Athens, Greece. Address correspondence to Clio P. Mavragani, MD, De- itary gland or a primary adrenal defect (7). Antibodies partment of Experimental Physiology, School of Medicine, University to 21-hydroxylase (anti-21[OH]), a major adrenal auto- of Athens, M. Asias 75, Athens, Goudi 1152, Greece. E-mail: antigen, are currently used to identify patients at in- [email protected]. Submitted for publication March 1, 2012; accepted in revised creased risk of developing autoimmune adrenalitis. form August 16, 2012. Autoimmune adrenalitis is the most common cause of 4066 ADRENAL AUTOIMMUNITY IN PRIMARY SS 4067 adrenal insufficiency in developed countries and is char- volvement (documented by biopsy showing changes compati- acterized by lymphocytic infiltration and destruction of ble with primary biliary cirrhosis in the setting of increased the adrenal glands. In cross-sectional studies, the prev- levels of liver enzymes or antimitochondrial antibodies), pal- pable purpura, vasculitis, peripheral neuropathy (verified by alence of autoantibodies against 21(OH) in patients with nerve conduction studies), lymphoma development (diagnosed Addison’s disease has been estimated to range from by histologic assessment), anti-Ro/SSA and/or anti-La/SSB 65% to 95%. This frequency tends to be higher in autoantibodies, rheumatoid factor (RF), low C3 and C4 levels, patients with shorter disease duration, declining over the cryoglobulinemia, hypergammaglobulinemia (total gamma Ͼ years to a frequency of 60% after 35 years of followup. globulin levels 2 gm/liter), and leukopenia (white blood cell count Ͻ4,000/mm3). Due to common underlying histopatho- In individuals with evidence of diseases other than logic features, the combined presence of interstitial nephritis, adrenal organ–specific autoimmune diseases, the pres- peribronchial disease, and autoimmune cholangitis was re- ence of antibodies to 21(OH) has been shown to predict corded as periepithelial involvement (1). progression to overt autoimmune failure over 6 years in The study was approved by the Ethics Committee of ϳ15% of patients (8). However, there is currently no Laikon General Hospital and the General Hospital of Athens information regarding the presence of antibodies against “G. Gennimatas.” All patients provided informed consent prior to study entry. adrenal antigens and the effect that their presence may Minor salivary gland (MSG) biopsy. Available MSG exert on adrenal function in patients with primary SS. biopsy samples (n ϭ 7) from patients with primary SS (all In the present study, we aimed to evaluate the women; ages 48–65 years at time of biopsy) were examined at prevalence of antibodies to 21(OH) in a cohort of the Department of Pathophysiology of the School of Medicine patients with primary SS and to investigate whether at the University of Athens, as a routine part of the diagnostic their presence could be related to evidence of primary evaluation for SS. A focus score was determined for each MSG biopsy sample, as previously described (9). adrenal insufficiency. Additionally, we sought to explore Anti-21(OH) antibody estimation. Anti-21(OH) anti- potential associations between anti-21(OH) titers and bodies were assessed using a commercial radioimmunoassay clinical, serologic, and histopathologic disease para- (21-OH Antibody Kit; RSR Ltd.). Briefly, calibrators, control specimens, and patient specimens were allowed to interact meters, including B cell–activating cytokine gene expres- 125 sion in salivary gland tissue. overnight with highly purified recombinant I 21-OH. After overnight incubation, solid-phase protein A was added to precipitate antibody-bound labeled 21(OH). The cutoff for PATIENTS AND METHODS positivity was 1 units/ml, according to the manufacturer’s instructions. Study groups. We studied 63 consecutive patients with Short Synacthen test. The secretory cortisol reserve of primary SS, who fulfilled the American–European Consensus the adrenal cortex was evaluated after adrenal stimulation Group criteria (9) and were followed up at the University of using synthetic ACTH (tetracosactide [Synacthen]). Briefly, Athens School of Medicine, Department of Pathophysiology 250 ␮g of Synacthen was injected intravenously, and blood and at the General Hospital of Athens “G. Gennimatas,” cortisol measurements were obtained at 0, 30, and 60 minutes Department of Rheumatology. Thirty-two patients with AITD in 11 patients with primary SS (5 of the 11 patients had positive (mainly Hashimoto thyroiditis) followed up at the Outpatient serum anti-21[OH] antibodies, and 6 age- and sex-matched Endocrinology Clinic at the University of Athens School of patients with negative anti-21[OH] antibodies served as con- Medicine Department of Pathophysiology, as well as 20 trols). Patients with past or current exposure to corticosteroids healthy controls, were also studied. The age and sex composi- were excluded from adrenal function testing. A normal re- tion was similar in all 3 groups. sponse to the test was defined as a serum cortisol concentra- Serum samples were collected during routine follow- tion of Ͼ500 nmoles/liter at 30 or 60 minutes poststimulation up visits and stored at Ϫ20°C until assayed. Clinical, serologic, (10). The percentage increase in the cortisol level from base- and histopathologic features were recorded after thorough line was also determined, defined as the ratio between the review of chart notes. The following features were noted: absolute increment in cortisol levels and the baseline cortisol arthralgias, arthritis, oral and ocular dryness (documented by levels (ϫ100). The Synacthen test was performed at 9:00 AM in subjective and objective measures), salivary gland enlarge- all patients. ment, Raynaud’s
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