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Autoimmune/Inflammatory Syndrome Induced by Adjuvants and Sjögren's

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Autoimmune/Inflammatory Syndrome Induced by Adjuvants and Sjögren's TH THE 4 ISRAEL-ITALY MEETING IMAJ • VOL 18 • march-aPrIL 2016 Autoimmune/Inflammatory Syndrome Induced by Adjuvants and Sjögren’s Syndrome Serena Colafrancesco MD1, Carlo Perricone MD1 and Yehuda Shoenfeld MD FRCP2 1Rheumatology Unit, Department of Medicine, Sapienza University of Rome, Rome, Italy 2Zabludowicz Center for Autoimmune Diseases, Sheba Medical Center, Tel Hashomer, affiliated with Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel A wide range of symptoms have been described, including Abstract: Sjögren’s syndrome (SS), a chronic systemic autoimmune myalgia, myositis, arthralgia, neurologic manifestations, fever, inflammatory condition involving the exocrine glands, has dry mouth, cognitive alterations, as well as chronic fatigue syn- been suggested to be part of the spectrum of the Autoimmune/ drome. In order to diagnose ASIA, major and minor criteria inflammatory Syndrome Induced by Adjuvants (ASIA). ASIA have been suggested, including the above mentioned symptoms incorporates an umbrella of clinical conditions including as well as the presence of autoantibodies or antibodies directed siliconosis, macrophage myofasciitis syndrome, and post- at the suspected adjuvant or specific HLA (i.e., HLA DRB1, vaccination phenomena that occur after the exposure to a HLA DQB1) [1]. For these conditions to develop, the existence substance, namely the adjuvant. Interestingly, SS and ASIA of a predisposing genetic background as well as different exter- share several common features. Firstly, a shared pathogenic nal or endogenous environmental agents are necessary. The mechanism involving a disruption of the immune system balance, with B cell proliferation, cytokine production and tissue trigger role of external factors is essential in this setting; several infiltration, has been proposed. Patients with ASIA often present of them have been recognized and are included in a group of clinical features resembling those of SS; dry mouth and dry eyes agents termed the “exposome” [3]. The evidence of a predispos- have also been included in the proposed classification criteria ing genetic background not only provides an explanation for for ASIA. Finally, several case reports have suggested that the rarity of ASIA syndrome but also clarifies why physicians both vaccines and silicone may trigger the development of SS. should be aware of the existence of this possible complication Unveiling these common pathways will contribute considerably following vaccine exposure in specific individuals [4]. to our understanding and management of both conditions. The most common agents acting as adjuvants are silicone, IMAJ 2016; 18: 150–153 aluminum, pristane, and infection. One of the principal mech- KEY words: autoimmune/inflammatory syndrome induced by adjuvants anisms of action is represented by molecular mimicry. Basically, (ASIA), Sjogren’s syndrome (SS), vaccine, autoimmunity, molecular mimicry refers to the way in which the immune adjuvants response, initially directed against bacterial or viral antigens, can target host molecules sharing sequence homology or structural similarities with microbial epitopes [2]. Other mechanisms of actions have also been described, including B cell polyclonal n 2011 a new term, “Autoimmune/inflammatory Syndrome activation, bystander activation (which enhances cytokine pro- I Induced by Adjuvants” (ASIA) was coined to illustrate a spec- duction and induces the expansion of autoreactive T cells), and trum of clinical conditions sharing similar signs or symptoms epitope spreading whereby invading antigens accelerate the local [1]. These disorders – including siliconosis, Gulf War Syndrome activation of antigen-presenting cells and the over-processing (GWS), macrophage myofasciitis syndrome (MMF), sick build- of antigens [4]. ing syndrome (SBS), and post-vaccination phenomena – all Aluminum is one of the principal adjuvants used in vaccine develop following the exposure to a common denominator: formulation and may be responsible for the development of the adjuvant. The adjuvant, defined as “any substance that acts ASIA syndrome. It seems that its ability to behave as an adju- to accelerate, prolong, or enhance antigen-specific immune vant might be related to evidence that aluminum salts seem to response” [2], has the property to boost the immune response both induce the activation of dendritic cells and complement without having any specific antigenic effect itself. Because of components and increase the level of chemokine secretion at this property, the adjuvant is considered a key factor able to the injection site [5]. stimulate the onset of such diseases. The clinical conditions Also, exposure to silicone is believed to boost the immune included in the ASIA spectrum represent immune mediated response. Silicone is considered an inert material, and for this disorders that usually appear following a chronic stimulation reason has been incorporated in different medical products of the immune system by agents with adjuvant characteristics. and devices such as breast implants. Following a prosthesis 150 TH IMAJ • VOL 18 • march-aPrIL 2016 THE 4 ISRAEL-ITALY MEETING break, local cutaneous inflammation, regional lymphadenopa- herpes virus (HHV) 6, 7 and 8. These viruses are able to induce thy and silicone granuloma have been reported; also described activation of the innate immune system via TLR pathways, is the remission of these conditions after implant removal. It which consequently stimulate the production of chemokines seems that silicone implants trigger a specific antigen-driven and cytokines whose expression is up-regulated in labial sali- local immune response through activated Th1/Th17 cells [6]. vary glands, plasma and peripheral blood cells of SS patients. Moreover, patients with severe immune mediated reactions Therefore, it should not be surprising for SS to develop, albeit to implanted silicone devices were found to have increased rarely, following the administration of a vaccine [11]. immunoglobulin G in the surrounding tissue and higher levels of anti-silicone antibodies [7]. These findings suggest a possible SJÖGREN’S syNDROME AND vacciNes adjuvant action of silicone. Some studies in the literature have reported the possible onset Interestingly, most of the clinical features included in the of SS following vaccine exposure [Table 1]. According to the ASIA criteria, such as dry mouth and/or dry eyes, myalgia and/ recommendation on vaccine administration in patients with or muscle weakness, arthralgia and/or arthritis and fatigue, are rheumatic diseases [12], several vaccines should be strongly typical manifestations of patients with Sjögren’s syndrome (SS). considered in SS (such as the influenzae, the 23-valent poly- For this reason, it would be interesting to analyze the possi- saccharide pneumococcal, herpes zoster, and tetanus toxoid ble connection between these two disorders, which might be vaccinations). However, other vaccines including Bacillus related to each other from both a pathogenic and clinical point Calmette Guérin (BCG), hepatitis A and/or B and human of view. papillomavirus, should be avoided or considered only in selected patients. PatHOGENesis OF SJÖGREN’S syNDROME AND ADJUvaNts There are several possible links between SS and vaccines. SS is a chronic autoimmune inflammatory disease character- Adjuvants may act by targeting the antigen-presenting cells via ized by the presence of lymphocyte infiltrates in the salivary TLRs, NOD-like receptors (NLRs), RIG-I-like receptors and glands. Once started, the ongoing inflammatory reaction leads C-type lectin receptors. The downstream signaling leads to the to a progressive glandular dysfunction with consequent onset activation of transcription factors such as NF-kB and IRF3, of sicca symptoms. In some patients extra-glandular mani- finally inducing the production of cytokines and chemokines festations occur, leading to life-threatening complications in involved in priming, expansion and polarization of the immune rare cases. A key role is played by the activated epithelium, response. Aluminum, one of the most commonly used adju- and several studies over the years have underlined the very vants, is able to trigger the activation of NLRP3 inflammas- high expression of the type I interferon (IFN) pathway in both ome signaling, leading to the production of pro-inflammatory peripheral blood mononuclear cells and minor salivary gland cytokines (IL-1b, IL-18) via caspase-1 activation [13]. Recently biopsies, supporting a role for the IFN ‘signature’ in the patho- investigated was the role of an aluminum-based adjuvant genesis of the disease. As mentioned above, the activated epi- (alum) in the induction of SS-like disorder in the genetically thelium is indispensable for disease onset, and the expression susceptible NZM2758 female strain that develops reduced sal- by these cells of Toll-like receptors (TLRs) and MHC class I ivary gland function and sialadenitis following injection with and II molecules is essential for the presentation of autoantigen incomplete Freund’s adjuvant [14]. The alum-treated mice and production of pro-inflammatory cytokines. Among these showed a persistent and significant reduction in salivary gland molecules, the production of B cell-activating factor (BAFF) function, an increased submandibular salivary gland inflamma- by epithelial cells greatly contributes to the stimulation of the tion, and the production of antinuclear antibodies as compared adaptive immune system and the potential development
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