290 The Burden of Systemic Disease in Patients with Primary Sjögren’s Syndrome (original) (raw)
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Arthritis Care & Research, 2012
A total of 1,927 participants in the SICCA registry were studied, including 886 participants who met the 2002 American-European Consensus Group (AECG) criteria for primary SS, 830 "intermediate" cases who had some objective findings of primary SS but did not meet AECG criteria, and 211 control individuals. We studied the prevalence of immunologic and hematologic laboratory abnormalities, specific rheumatologic examination findings, and physicianconfirmed thyroid, liver, and kidney disease, as well as lymphoma among SICCA participants. Results. Laboratory abnormalities, including hematologic abnormalities, hypergammaglobulinemia, and hypocomplementemia, frequently occurred among primary SS cases and were more common among the intermediate cases than among control participants. Cutaneous vasculitis and lymphadenopathy were also more common among primary SS cases. In contrast, the frequency of physician-confirmed diagnoses of thyroid, liver, and kidney disease and lymphoma was low and only primary biliary cirrhosis was associated with primary SS case status. Rheumatologic and neurologic symptoms were common among all SICCA participants, regardless of case status. Conclusion. Data from the international SICCA registry support the systemic nature of primary SS, manifested primarily in terms of specific immunologic and hematologic abnormalities. The occurrence of other systemic disorders among this cohort is relatively uncommon. Previously reported associations may be more specific to select patient subgroups, such as those referred for evaluation of certain neurologic, rheumatologic, or other systemic manifestations.
Clinical Rheumatology, 2020
Objectives This study compared the clinical and serological characteristics of seronegative and seropositive primary Sjögren syndrome (pSS) and examined whether current classification criteria for pSS cover seronegative pSS. Methods The study group comprised 375 patients (341 women and 34 men) diagnosed with pSS. A clinical diagnosis by an expert rheumatologist was considered the "gold standard" for the diagnosis of pSS. The clinical and serological characteristics of the patients were retrospectively collected from hospital medical files. Results Fifty-eight of the 375 pSS patients (15.5%) were seronegative for ANA, RF, anti-Ro, and anti-La autoantibodies. Seronegative pSS was diagnosed based on lymphocytic infiltrations in lip biopsy samples. There were no statistically significant differences in terms of patient age, age at diagnosis, sex distribution, clinical features, and laboratory findings between seronegative and seropositive pSS. The frequency of hypergammaglobulinemia was higher in seropositive pSS. The 2016 ACR/ULAR criteria best covered most seronegative pSS cases (84.5%). For seronegative pSS, the agreement between the 2002 AECG, 2012 ACR, and 2016 ACR/EULAR criteria was relatively low. Conclusions The clinical features of seronegative pSS (i.e., a lack of four autoantibodies in serum) were similar to those of seropositive pSS. The current classification criteria for pSS should not be used in the diagnosis of seronegative pSS, as the agreement between the different sets of criteria was low, and some patients fell outside the classification. Further clinical and laboratory studies are needed to identify the features that distinguish seronegative pSS. Keywords Anti-La. Antinuclear antibody (ANA). Anti-Ro. Classification criteria. Rheumatoid factor (RF). Sjögren's syndrome Key Points • Approximately 15% of the pSS patients were seronegative for ANA, RF, anti-Ro, and anti-La autoantibodies. • Seronegative pSS was diagnosed based on lymphocytic infiltrations in lip biopsy samples. • The clinical features of seronegative pSS were similar to those of seropositive pSS. • The current classification criteria for pSS should not be used in the diagnosis of seronegative patients, as the agreement between the different sets of criteria was low, and some patients fell outside the classification.
Rheumatology (Oxford, England), 2014
The aims of this study were to describe the clinical presentation of primary SS (pSS) in a large cohort of patients by assessing the prevalence of the patient subgroups at high risk for severe extraglandular manifestations and to explore the influence of the patients' serological profile on disease severity and on immunosuppressive drug utilization. Cumulative demographic, clinical, serological, histological and therapeutic data of 1115 pSS patients were retrospectively evaluated. Independent serological markers for glandular and extraglandular disease manifestations were identified by logistic regression. The cohort included 1115 (1067 female, 48 male) pSS patients. Severe extraglandular manifestations were detectable in 15% of the patients and were represented by active synovitis (11%), axonal sensory-motor neuropathy (2%), severe leucocytopenia (14%), cutaneous vasculitis (6%) and non-Hodgkin's lymphoma (4.5%). We found that low C3/C4, hypergammaglobulinaemia, RF and cryo...
Clinical Reviews in Allergy & Immunology, 2009
The current 2002 classification criteria do not cover the broad clinical and immunological heterogeneity of primary Sjögren syndrome (SS), since five of the six criteria focus exclusively on glandular involvement and the remaining criterion is the mandatory presence of anti-Ro/La antibodies. The aim of this study was to analyze the clinical features of patients with a well-established diagnosis of primary SS who do not fulfill the 2002 classification criteria. Five hundred seven patients diagnosed with primary SS (1993 criteria) were consecutively included and followed up. Two hundred twenty-one (44%) patients did not fulfill the 2002 criteria. These patients were older at diagnosis (p<0.001) and had a lower frequency of parotid enlargement (p=0.002), fever (p=0.041), arthritis (p=0.041), vasculitis (p=0.050), peripheral neuropathy (p=0.002), cranial nerve involvement (p=0.015), raised erythrocyte sedimentation rate (ESR) levels (p<0.001), anemia (p<0.001), leukopenia (p=0.037), hypergammaglobulinemia (p<0.001), positive rheumatoid factor (RF; p=0.002), and cryoglobulinemia (p=0.049) in comparison with those fulfilling 2002 criteria. However, there were no significant differences in the prevalence of sicca features, diagnostic tests, overall systemic involvement, antinuclear antibodies, complement levels, development of B-cell lymphoma, or survival. Patients with anti-Ro antibodies had the highest frequencies of systemic features, hematological abnormalities, and altered immunological markers. In conclusion, patients fulfilling the 2002 criteria, who have either a specific histological diagnosis (lymphocytic infiltration) or highly specific autoantibodies (Ro/La), might well be considered to have Sjögren "disease." In contrast, etiopathogenic mechanisms other than lymphocytic-mediated epithelial damage could be involved in patients with negative Ro and negative biopsy, in whom the term Sjögren "syndrome" seems more adequate.
Primary Sjögren’s Syndrome: Current Pathophysiological, Diagnostic and Therapeutic Advances
Autoimmune Disorders - Current Concepts and Advances from Bedside to Mechanistic Insights, 2011
The diagnosis of SS is currently essentially based on the American-European criteria. These criteria, 6 in total, include 2 subjective and 4 objective criteria. The subjective criteria are www.intechopen.com Autoimmune Disorders-Current Concepts and Advances from Bedside to Mechanistic Insights 42 ocular and oral symptoms, while the objective criteria are ocular signs, histopathology, salivary gland involvement, and autoantibodies. Experts have recommended making the diagnosis of SS when 4 of the 6 criteria are present, as long as histopathology or serology is positive, or when 3 of any of the 4 objective criteria are present (Vitali et al., 2002). It necessary to bear in mind that the most frequent symptoms of SS include the triad of fatigue, polyarthralgia and sicca symptoms, which are often commonly found in the general population and aging population. Many drugs have anti-cholinergic properties, which complicate the diagnostic process of SS. Exclusion criteria for the diagnosis of SS include the presence of hepatitis C and HIV viruses, sarcoidosis, prior cervical radiation, lymphoma, graft versus host disease and the use of anti-cholinergic drugs (Vitali et al., 2002). 2.1 Ocular symptoms Ocular symptoms are taken into consideration when patients complain about troublesome dry eyes, recurrent sensation of sand or gravel in the eye, or the use of tear substituent. 2.2 Oral symptoms Oral symptoms are considered pertinent when patients experience daily feeling of dry mouth, recurrent or persistent swollen salivary glands, or frequent drinking to facilitate dry food swallowing. 2.3 Ocular signs Evidence of ocular involvement relies on positive Schirmer's test (<5mm/min) or positive ocular dye score (score > 4 according to the van Bijsterveld score; Van Bijsterveld, 1969). 2.4 Histopathology A histological sign is considered positive when minor salivary gland biopsy show a focus score >1 (more than 50 lymphocytes per 4 mm 2 of tissue). 2.5 Salivary gland involvement Objective evidence of salivary gland involvement relies on low unstimulated saliva flow (< 1.5ml/15 min), abnormal sialography, or altered salivary scintigraphy. 2.6 Autoantibodies Serological signs are considered positive when the presence of antibodies to either Ro (SSA) or La (SSB) or both are detected in the serum. 2.7 New diagnostic tools To prevent excessive prescriptions of exams for establishing diagnosis of SS, newer diagnostic tools have been validated. However, these tools have not yet been included in the American-European diagnosis criteria of SS. For example, ultrasonography of salivary glands might prove to be useful to detect anatomical changes in parotid and submandibular glands, with similar diagnostic ability to sialography. Detection of hypoechoic areas, echogenic streaks, cysts and irregular gland margins are highly suggestive of SS (Tagaki et al., 2010). Parotid MRI can also prove to be an adjunct diagnostic tool to detect heterogeneity in salivary glands and specific cystic lesions (Roberts et al., 2008).
Rheumatology (Oxford, England), 2015
To reach a European consensus on the definition and characterization of the main organ-specific extraglandular manifestations in primary SS. The EULAR-SS Task Force Group steering committee agreed to approach SS-related systemic involvement according to the EULAR SS Disease Activity Index (ESSDAI) classification and proposed the preparation of four separate manuscripts: articular, cutaneous, pulmonary and renal ESSDAI involvement; muscular, peripheral nervous system, CNS and haematological ESSDAI involvement; organs not included in the ESSDAI classification; and lymphoproliferative disease. Currently available evidence was obtained by a systematic literature review focused on SS-related systemic features. The following information was summarized for articular, cutaneous, pulmonary and renal involvement: a clear, consensual definition of the clinical feature, a brief epidemiological description including an estimate of the prevalence reported in the main clinical series and a brief l...
An unusual clue in the diagnosis of primary Sjogren’s syndrome
Revista română de reumatologie, 2023
Sjogren's syndrome (SSj) is a chronic autoimmune disease mainly targeting the exocrine glands, but sometimes associating extra-glandular manifestations. Xerosis, purpura, Raynaud's phenomenon, cutaneous vasculitis, annular erythema are the main forms of skin involvement. A 26-year-old female patient was admitted for diffuse erythematous rash and angioedema, xerophthalmia and symmetrical arthralgia of hand joints. Anti-nuclear antibodies, anti-SSA and anti-Ro52 antibodies were identified, Schirmer's test was positive, thus the diagnoses of primary SSj and associated urticarial vasculitis were established. Treatment with oral methylprednisolone, azathioprine and hydroxychloroquine was initiated, with favourable response over the next week. Patients with primary SSj that develop cutaneous vasculitis, lymphadenopathies or lymphopenia may be at risk for additional extra-glandular manifestations, including non-Hodgkin lymphoma.