The overlap of Sjögren's syndrome with other systemic autoimmune diseases

OBJECTIVE: To analyze the main diagnostic problems caused by the overlap between Sj?gren's syndrome (SS) and other systemic autoimmune diseases (SAD). METHODS: We performed a MEDLINE search for articles published between January 1966 and December 2005 that specifically analyzed the overlap between SS and other SAD. We identified a list of diagnostic problems in patients with primary SS who had features considered typical of other SAD. RESULTS: Clinically, the main diagnostic problems occur in SS patients presenting with arthritis, Raynaud phenomenon, cutaneous features (subacute cutaneous lupus erythematosus, purpura, livedo reticularis, erythema nodosum), interstitial pulmonary disease, and cytopenias (leukopenia, thrombocytopenia). Immunologically, antiphospholipid antibodies (aPL) and antineutrophil cytoplasmic antibodies (ANCA) are the most frequent atypical autoantibodies found in primary SS, with a prevalence ranging between 10 and 20%. However, coexisting antiphospholipid syndrome or systemic vasculitis is only detected in around 10% of SS patients with aPL or ANCA. Anti-DNA and anticentromere antibodies have a prevalence of 5 to 10%, but are more closely related to clinical and/or laboratory data suggestive of associated systemic lupus erythematosus and limited systemic sclerosis, respectively, leading to the fulfillment of classification criteria for these diseases in more than 25% of cases. CONCLUSION: The wide variety of clinical and immunological manifestations of patients with primary SS often overlap with other SAD, making the differentiation between primary SS, SS associated with SAD, and SS-like presentations of some other SAD difficult. This overlap suggests that the current classification criteria are useful in differentiating between autoimmune and non-autoimmune processes but fail to clearly differentiate among SAD.     

Anti-pm/scl antibodies in connective tissue disease: Clinical and biological assessment of 14 patients

INTRODUCTION: Anti-PM/Scl antibodies (Anti-PM/Scl) represent a rarely encountered type of antinuclear antibodies. They have mainly been reported in association with idiopathic inflammatory myositis - systemic sclerosis overlap syndromes (also called scleromyositis or sclerodermatomyositis) but also with polymyositis, dermatomyositis and systemic sclerosis without features of overlap syndromes. Studies concerning characteristics of patients with anti-PM/SCl are rare and include small numbers of patients. PATIENTS AND METHODS: Retrospective review of clinical and biological characteristics of 14 patients with anti-PM/Scl in two University Hospitals: one in Belgium (Erasme Hospital, Bruxelles) and one in France (Hautepierre Hospital, Strasbourg). RESULTS: Seven patients were identified in Erasme and 7 in Strasbourg: 5 with systemic sclerosis-(dermato)myositis overlap syndromes, 4 with dermatomyositis, 1 with polymyositis, 3 with systemic sclerosis, 1 with primary Sj?gren's syndrome. The most frequently observed clinical characteristics (85% of patients) were: pulmonary interstitial disease and arthralgia or arthritis. No patient of our series died or developed cancer (mean follow-up:6.1 years). CONCLUSIONS: Our study failed to identify an homogeneous clinical pattern in patients with anti-PM/Scl, except for 2 characteristics shared by 85% of the patients. This lack of homogeneity is in agreement with preceding literature. We confirm the favourable prognosis associated with the presence of anti-PM/Scl, despite the high incidence of interstitial pulmonary disease. The absence of cancer associated with presence of anti-PM/Scl represents a partial explanation. Finally, we report herein the second case of primary Sj?gren's syndrome associated with anti-PM/Scl.     

Adverse drug reactions in Sj?gren's syndrome. Frequent allergic reactions and a specific trimethoprim-associated systemic reaction.

Trimethoprim-associated systemic reactions, including aseptic meningitis, have been reported to be very rare adverse drug reactions. Patients with Sj?gren's syndrome have been overrepresented, but no epidemiological surveys of the reaction have been conducted. To study the overall frequency of adverse drug reactions, and especially trimethoprim-associated reactions, we interviewed 85 primary Sj?gren's syndrome patients and compared the results with those of 45 similarly interviewed osteoarthritis patients. Antimicrobial allergy was more common among Sj?gren's syndrome patients than in osteoarthritis patients (46% vs. 27%). Eleven Sj?gren's syndrome patients (13%), but no osteoarthritis patient, had experienced at least a partial, non-allergic systemic reaction with trimethoprim. Of them five (6%) had had a full-blown systemic reaction including both chills/fever and headache/backache and at least one of the following: malaise, vomiting, dizziness, confusion or meningeal irritation. Our findings confirm that allergic reactions to antimicrobials are frequent in Sj?gren's syndrome. In addition to allergic reactions Sj?gren's syndrome patients are prone to a specific trimethoprim-associated systemic reaction. This should be remembered when prescribing antimicrobials.     

Ear-nose-throat manifestations of autoimmune rheumatic diseases

Ear-nose-throat (ENT) manifestations of connective tissue disorders represent a diagnostic challenge for clinicians as they often constitute the initial sign of an otherwise asymptomatic autoimmune disease. Moreover, in patients with known autoimmune rheumatic diseases, ENT manifestations can be overlooked. Hearing disturbances may be seen in patients with systemic lupus erythematosus, Wegener's granulomatosis, relapsing polychondritis, polyarteritis nodosa, Cogan's syndrome, Sj?gren's syndrome, and less frequently in Churg-Strauss syndrome and Adamantiades-Beh?et's disease. Nose and paranasal sinuses are variably affected during the course of Wegener's granulomatosis, Churg-Strauss syndrome, relapsing polychondritis and sarcoidosis. Recurrent mucosal ulcerations are common in systemic lupus erythematosus and Adamantiades-Beh?et's disease. Xerostomia is a common feature of primary and secondary Sj?gren's syndrome; salivary gland enlargement may be also seen in these patients, as well as in patients with sarcoidosis. The cricoarytenoid joint can be involved during the course of rheumatoid arthritis, ankylosing spondylitis and gout; osteoarthritic changes have also been described. Motility disorders of the upper and/or the lower portions of the esophagus have been reported in patients with dermatomyositis/polymyositis, systemic sclerosis and systemic lupus erythematosus. Trigeminal nerve dysfunction may occur in patients with Sj?gren's syndrome, systemic sclerosis, systemic lupus erythematosus and mixed connective tissue disease. Peripheral facial nerve palsy has been described to complicate the course of Sj?gren's syndrome and sarcoidosis.     

Anti-cyclic citrullinated peptide antibodies in hepatitis C virus associated rheumatological manifestations and Sjogren's syndrome

OBJECTIVE: To investigate the diagnostic reliability of anti-CCP antibodies (anti-CCP Ab) in distinguishing hepatitis C virus (HCV) associated rheumatological manifestations and Sj?gren's syndrome from rheumatoid arthritis. METHODS: 147 HCV infected patients (HCV RNA positive) were compared with 64 patients with definite rheumatoid arthritis in a retrospective study. Anti-CCP Ab were detected using the Immunoscan ELISA kit (second generation) and rheumatoid factor (RF) by the FIDIStrade mark Rheuma kit. RESULTS: Among the 147 HCV infected patients (77 women; mean (SD) age 58 (16) years), 77 (52%) had a mixed cryoglobulin (MC), 38 (26%) an MC associated systemic vasculitis, 35 (24%) arthralgia/arthritis, and seven (5%) definite Sj?gren's syndrome. HCV infected patients with arthralgia were more often RF positive than those without arthralgia (54% v 27%; p = 0.003), but less often than patients with rheumatoid arthritis (54% v 81%; p = 0.009). Anti-CCP Ab were detected in only two HCV infected patients with arthralgia (5.7%), in none without arthralgia or with Sj?gren's syndrome, and in 78% of patients with rheumatoid arthritis. With a specificity of 93.5% and a positive predictive value of 96% for rheumatoid arthritis, anti-CCP Ab were the most specific biological marker. CONCLUSIONS: Anti-CCP antibodies are very rarely found in HCV infected patients with rheumatological manifestations or Sj?gren's syndrome. They are reliable serological markers to distinguish these from patients with rheumatoid arthritis.     

Anticentromere antibody — clinical associations

The objective of this study was to determine the clinical features of 44 patients with anticentromere antibody (ACA) positivity. We undertook a retrospective review of 44 ACA-positive patients (1 male and 43 females with a mean±SD age of 53.6±12.2 years). There were 25 patients with limited systemic sclerosis, 12 with Raynaud's disease, 2 with Sjögren's syndrome, 2 with systemic lupus erythematosus and 3 with polyarthritis. ACA was more frequently found in patients affected by limited systemic sclerosis with mild visceral involvement and in patients with Raynaud's disease. Moreover, ACA was detected in other connective tissue diseases that were characterized by an atypical autoantibody profile.     

Anti-SSB/La antibodies in Sj?gren's syndrome and related autoimmune diseases. Results of a quantitative immunoassay using a highly purified antigen

A quantitative immunoassay using a highly purified antigen from HeLa cells has allowed us to detect antibodies to SSB/La in 11/20 patients with primary Sj?gren's syndrome (SS), 15/33 with SLE, and 11/35 with progressive systemic sclerosis (PSS). However, positive results were found in only 2/12 patients with idiopathic Raynaud's disease and 2/20 with rheumatoid arthritis, including 4 with secondary SS. Anti-SSB/La were associated with extraglandular manifestations in primary SS, and with a diffuse sclerodermic pattern in PSS. In SS, SLE and PSS, a positive anti-SSB/La test was strongly associated with nodal or spleen enlargement and with an increased level of gamma-globulins. A direct association was also found with positive tests for rheumatoid factors, anti-SSA/Ro and anti-Scl 70. An inverse relationship was however found with anti-nRNP +/- Sm and anticentromere antibodies. Our data suggest that anti-SSB/La antibodies can be regarded as a marker of B-lymphocyte activation in patients with either primary SS, SLE or PSS.     

Sensitivity and specificity for primary Sjögren's syndrome of IgA and IgG anti-alpha-fodrin antibodies detected by ELISA

OBJECTIVE: To investigate the sensitivity and specificity of anti-alpha-fodrin antibodies in patients with primary Sj?gren's syndrome (pSS). METHODS: IgA and IgG anti-alpha-fodrin antibodies were measured in the sera of 80 patients with pSS, 60 blood donors matched for age and sex, 50 patients with systemic lupus erythematosus (SLE), 30 with rheumatoid arthritis (RA), 20 with systemic sclerosis (SSc), and 10 with polymyositis or dermatomyositis (PM/DM) by an ELISA method employing recombinant human alpha-fodrin as antigen. RESULTS: The sensitivity of IgA and IgG anti-alpha-fodrin antibodies for pSS was 32.50% and 21.25%, respectively. When the prevalence of these antibodies in patients with SLE, RA, SSc, and PM/DM was evaluated, we observed specificity of these antibodies of 68.18% and 79.09%, respectively. The sensitivity and specificity for pSS of the combined determination of IgA and IgG anti-alpha-fodrin antibodies were 40% and 58.18%, respectively. CONCLUSION: The prevalences of IgA and IgG anti-alpha-fodrin antibodies in our patients with pSS and other chronic autoimmune diseases have induced us to doubt their use as diagnostic markers of pSS.     

Vasculitis in systemic sclerosis: association with Sj?gren's syndrome and the CREST syndrome variant

We describe 7 patients with established systemic sclerosis who developed clinical evidence of vasculitis 1 to 33 (mean 12.7) years after the first symptoms of scleroderma. Six had the CREST variant of systemic sclerosis and also had features of Sj?gren's syndrome (SS). Five of 6 patients tested had serum anti-SSA (Ro) antibodies. Vasculitis presented primarily as cutaneous lesions with ulceration and/or mononeuritis multiplex, and 6 patients had severe systemic manifestations. Vasculitis was histopathologically documented in 6 cases in biopsies of skin (4 of 4), muscle (2 of 3) and sural nerve (3 of 3). Patients with systemic sclerosis with CREST syndrome and SS appear to be at increased risk to develop vasculitis.     

A clinical analysis of primary Sjögren's syndrome with anticentromere antibodies

OBJECTIVE: To investigate the clinical manifestations, immunological features and prognosis of primary Sj?gren's syndrome (pSS) with anticentromere antibodies (ACA). METHODS: Sixty pSS patients with ACA in our hospital between 1985 and 2006 were screened retrospectively and compared with those without ACA. RESULTS: The mean age at the onset of pSS with ACA was higher than that of those without ACA [(48 +/- 11) yr vs (41 +/- 12) yr, P =0.000]. There was no difference in sex ratio, dry mouth, dry eyes and positive salivary gland biopsy between the two groups (P > 0.05). Compared with those without ACA, patients with ACA presented a higher prevalence of liver involvement (68.3% vs 37.0%, P = 0.000), while a lower prevalence of renal involvement (13.3% vs 30.9%, P = 0.009), neuropathy (1.7% vs 11.5%, P = 0.025) and hypergammaglobulinemia (20.8% vs 45.7%, P = 0.002). The difference was not significant between the two groups in Raynaud's phenomenon, articular involvement, myositis, hematologic involvement, lung involvement, and thyroiditis. While both groups showed the same prevalence of antinuclear antibody (ANA), the patterns of ANA-IF were different and the discrete speckled pattern was the most frequent in patients with ACA and occurred in 61.7%. Different from those without ACA, patients with ACA presented a lower prevalence of anti-SSA, anti-SSB, rheumatoid factor, and anti-U1RNP, while showed a higher prevalence of antimitochondrial antibodies (AMA) and AMA-M2. The most frequent cause of death was the complications associated with cirrhosis, notably bleeding varices (3/5 cases). CONCLUSION: Patients with ACA present a high risk of liver involvement. Because of the remarkable difference in the mean age of disease onset and also differences in systemic damage, immunological and antibody features, pSS with ACA may be a special subtype of pSS.     

抗着丝点抗体阳性原发性干燥综合征的临床特点

目的 通过分析抗着丝点抗体(ACA)阳性原发性干燥综合征(pSS)患者的临床特点,提高对本病的诊治水平.方法 回顾性分析北京协和医院风湿免疫科收治的60例ACA阳性pSS患者(ACA阳性组)的临床资料,并与同期收治的139例ACA阴性pSS者(ACA阴性组)相比较.结果 (1)ACA阳性组发病年龄晚于ACA阴性组[(48±11)岁比(41±12)岁,P=0.000],而2组在性别构成、口干、眼干、唇腺活检阳性率差异元统计学意义.(2)肝脏受累是ACA阳性组患者最常见的腺外脏器损害,其发生率远高于ACA阴性组(68.3%比37.0%,P=0.000),而肾脏受累(13.3%比30.9%,P=0.009)、神经系统受累(1.7%比11.5%,P=0.025)、高γ球蛋白血症的发生率(20.8%比45.7%,P=0.002)均低于ACA阴性组.(3)2组抗核抗体阳性率无差异,但ACA阳性组以散点型免疫荧光表型最为常见(占61.7%).与ACA阴性组相比,ACA阳性组中抗SSA抗体、抗SSB抗体、抗U1 RNP抗体的阳性率减低(P＜0.05),而抗线粒体抗体及其M2亚型的阳性率增高(P＜0.05).(4)ACA阳性组死亡5例,其中3例死于胃食管静脉曲张破裂出血.结论 对ACA阳性pSS者应警惕肝脏受累.由于ACA阳性pSS在发病年龄、腺外器官受累、免疫指标、自身抗体谱及预后方面均不同于经典pSS,故可能是pSS的一种独特亚型.     

Antibodies to CD4 in primary Sjögren's syndrome

OBJECTIVE: The purpose of this study was to examine whether antibodies against CD4 are present in patients with primary Sj?gren's syndrome, and to explore the possible correlation between these antibodies and the CD4+ T lymphocyte depletion that is seen in some Sj?gren patients. METHODS: Sera from 214 patients with primary Sj?gren's syndrome, 154 healthy blood donors, 38 age- and sex-matched controls without autoimmune disease, and 77 HIV-1-seropositive individuals were analysed by an enzyme-linked immunosorbent assay (ELISA) using recombinant soluble CD4 as the antigen. RESULTS: Anti-CD4 antibodies were observed more frequently in patients with Sj?gren's syndrome (12.6%) as compared with the control groups (0.6%) (P < 0.001), and at a level similar to that seen among the HIV-1 patients (13.0%). However, no correlation was found between the presence of anti-CD4 antibodies and CD4+ T lymphocytopenia in the Sj?gren patients. CONCLUSION: This is the first study that shows anti-CD4 antibodies in patients with primary Sj?gren's syndrome. The significance of these antibodies in the immunopathogenesis of Sj?gren's syndrome remains to be determined.     

Sj?gren's syndrome in systemic sclerosis. A clinical study of 26 patients

Features of Sj?gren's syndrome were sought in 26 patients with systemic sclerosis and in age- and sex-matched control subjects. The assessments included a structured history to establish symptoms of dry eyes and dry mouth. Schirmer's I and II tear tests. Rose Bengal staining with slit lamp microscopy of the eyes, measurement of basal and stimulatory salivary secretion. We measured sweat secretion rates from the skin. Salivary scintigraphy and skin biopsies were performed on the patients. Only one patient showed the complete picture of Sj?gren's syndrome with both clinical and investigational evidence of lacrimal and salivary gland involvement. A further patient had an abnormal Schirmer's II test and xerostomia with reduced salivary secretion and an abnormal scan, but no ocular symptoms and no keratoconjunctivitis sicca on ophthalmological examination. Two patients had reduced salivary flow and a dry mouth. A number of patients and control subjects showed various individual symptoms and signs of lacrimal and salivary disorders. These features alone are not sufficient for the diagnosis of Sj?gren's syndrome. There is a clear need to adopt strict criteria for diagnosing the condition. The association of Sj?gren's syndrome with systemic sclerosis seems doubtful and if it does occur it is very much less common than previously suggested.     

Optic neuropathy and central nervous system disease associated with primary Sj?gren's syndrome.

Three cases of optic neuropathy associated with primary Sj?gren's syndrome are reported. All three patients had clinical manifestations of primary Sj?gren's syndrome, although two of the patients did not report sicca symptoms at initial examination. Two patients had focal neurologic signs in addition to optic neuropathy. The differentiation of this syndrome of optic neuropathy, focal neurologic signs, and Sj?gren's syndrome from multiple sclerosis and antiphospholipid antibody syndrome is important for reasons of treatment and prognosis. This diagnostic differentiation was facilitated by positive tests for xerophthalmia and findings of positive minor salivary gland biopsy. High titers of antinuclear antibody, anti-SSA(Ro), and anti-SSB(La), and the absence of antiphospholipid antibodies provided additional help in the differential diagnosis. In 5 years of observation, none of the patients developed symptoms of multiple sclerosis or additional connective tissue disorders.     

Autoimmune thyroid disease is associated with a diagnosis of secondary Sjögren's syndrome in familial systemic lupus

BACKGROUND: Autoimmune thyroid disease is common in systemic lupus erythematosus (SLE). About 20% of patients with SLE have secondary Sj?gren's syndrome. METHODS: Families with more than one patient with SLE were identified. All patients met the revised classification criteria, although SLE-unaffected relatives were confirmed not to satisfy these criteria. Diagnosis of autoimmune thyroid disease and Sj?gren's syndrome was made on the basis of a review of medical records, interview and questionnaire administered to patients with SLE, and by a questionnaire administered to SLE-unaffected subjects. RESULTS: Of a total of 1138 patients with SLE, 169 had a diagnosis of Sj?gren's syndrome. Of these 50 (29.6%) patients also had autoimmune thyroid disease. Of the 939 patients with SLE with no diagnosis of Sj?gren's syndrome, 119 (12.7%) had autoimmune thyroid disease (chi2 = 20.1, p = 0.000009). There was no association of a diagnosis of hypertension with secondary Sj?gren's syndrome (42% vss 47%). Among 2291 SLE-unaffected relatives, 44 had diagnosed primary Sj?gren's syndrome and 16 (36.3%) of these also had autoimmune thyroid disease. 265 of 2247 (11.8%) subjects had autoimmune thyroid disease but no Sj?gren's syndrome (chi2 = 24.2, p<0.001). CONCLUSIONS: Autoimmune thyroid disease is found in excess among patients with SLE with a diagnosis of secondary Sj?gren's syndrome, as well as among their SLE-unaffected relatives with a diagnosis of primary Sj?gren's syndrome.     

Early identification of vascular damage in patients with systemic sclerosis

Vascular involvement in systemic sclerosis (SSc) plays a key role in the pathogenesis of fibrosis. We assessed arterial stiffness using a new echo-tracking technique in patients with SSc asymptomatic for cardiovascular diseases. We enrolled 22 patients (21 female, 63 ± 14 years) and 20 controls (12 female, 62 ± 3 years). Carotid intima-media thickness (IMT) was comparable between the 2 groups (1.1 ± 0.3 vs 1.0 ± 0.4 mm, P = ns), whereas the stiffness parameters were significantly increased in patients (β: 9.5 ± 4.2 vs 5.8 ± 1.1, P = .001; pulse wave velocity [PWV]: 6.5 ± 1.5 vs 5.2 ± 0.6 m/sec, P = .003). A correlation between stiffness parameters, anti-Scl-70 antibodies (β: r = .46, P = .03; PWV: r = .50, P = .02), and anticentromere antibodies (β: r = -.54, P = .020; PWV: r = -.53, P = .023) was found. Echo-tracking technique may be valuable in early identification of vascular involvement in patients with SSc.     

Raynaud's phenomenon in primary Sjögren's syndrome. Prevalence and clinical characteristics in a series of 320 patients

OBJECTIVE: To determine the prevalence of Raynaud's phenomenon (RP) in a large series of patients with primary Sj?gren's syndrome (SS) and to identify the clinical and immunological features related to its presence. METHODS: In a cross sectional study, we investigated 320 consecutive patients with primary SS (294 women, 26 men; mean age at onset 60 yrs, range 16-87 yrs). All patients fulfilled 4 or more of the diagnostic criteria for SS proposed by the European Community Study Group in 1993. Diagnosis of RP in patients with SS was defined as intermittent attacks of digital pallor and/or cyanosis in the absence of any other associated disease or anatomical abnormalities. RESULTS: RP was present in 40 (13%) patients. All were women, with a mean age of 57 yrs (range 18-78). RP preceded onset of sicca symptomatology in 18 (45%) patients. The main triggering factor was exposure to cold, which induced RP in all patients, while emotional stress was a factor in 12 patients, as was job related predisposition in 2. Fifteen (38%) patients required pharmacological treatment with calcium channel blockers (12 patients) or angiotensin converting enzyme inhibitors (2 patients) during colder months, and one patient required treatment with intravenous prostacyclin for ischemic complications. Compared with SS patients without RP, those with RP showed a higher prevalence of articular involvement (50 vs 31%; p = 0.031), cutaneous vasculitis (30 vs 11%; p = 0.003), antinuclear antibodies (95 vs 65%; p < 0.001), anti-Ro/SSA (59 vs 31%; p < 0.001) and anti-La/SSB antibodies (44 vs 20%, p = 0.003). CONCLUSION: We found RP in 13% of patients with primary SS, in almost half of whom RP was the first autoimmune symptomatology. These patients constituted a subset of SS with a higher frequency of some extraglandular features and positive immunological markers. The clinical course of RP seems to be milder in patients with primary SS than in those with other systemic autoimmune diseases such as systemic sclerosis, with no vascular complications and pharmacological treatment needed in only 40% of patients.     

Laboratory diagnostics of systemic autoimmune diseases. Part 1. Collagenoses

This is the first part of a series of articles on the laboratory diagnostics of rheumatic diseases and will consider the systemic autoimmune diseases lupus erythematosus, Sj?gren's syndrome, systemic sclerosis, dermato/polymyositis and mixed connective tissue disease (MCTD, SHARP syndrome). The basis for diagnostics is the presence of antinuclear antibodies (ANA). Initially, these antibodies are detected using a screening test. This must be followed by the identification of the patient's individual autoantibody specificities, which then yields important diagnostic clues. Disease activity may be monitored serologically by following the titers of selected autoantibodies and, in certain patients, by examining complement consumption.     

Autoantibodies to lipoprotein lipase and dyslipidemia in systemic lupus erythematosus

OBJECTIVE: To demonstrate the binding of bovine lipoprotein lipase (LPL) by IgG from sera obtained from patients with systemic lupus erythematosus (SLE) and other rheumatic diseases, and the relationship of anti-LPL to triglyceride levels in SLE. METHOD: Binding of LPL by IgG from sera obtained from patients with SLE and other rheumatic diseases was measured by an enzyme-linked immunosorbent assay technique. Lipid profiles for fasting blood samples obtained from SLE patients and control subjects were determined. RESULTS: Sera obtained from 105 patients with SLE were assessed for reactivity with LPL, and 49 (47%) of the results were positive. Sera obtained from patients with rheumatoid arthritis (RA) (n = 80), Sj?gren's syndrome (n = 30), polymyositis and dermatomyositis (n = 30), and progressive systemic sclerosis (n = 31) were also studied, and 10 (13%), 3 (10%), 12 (40%), and 13 (42%), respectively, were positive for reactivity with LPL. It was determined that all affinity-purified anti-double-stranded DNA (dsDNA) antibodies and 4 of 5 monoclonal anti-dsDNA antibodies bound to LPL. The binding of IgG depleted of anti-dsDNA to LPL indicates a second anti-LPL activity in SLE. Measurements of fasting lipid levels in SLE patients with anti-LPL revealed a strong positive correlation of antibody levels and total serum triglycerides, apolipoprotein B, and apolipoprotein E concentrations. CONCLUSION: Antibodies to LPL occurred in 47% of SLE patients and in a similar percentage of patients with polymyositis or systemic sclerosis. The prevalence of these antibodies was less in patients with RA or Sj?gren's syndrome. It is hypothesized that the elevated triglyceride levels in SLE patients are in part attributable to anti-LPL, and this lipid abnormality could contribute to the premature atherosclerosis known to be present in patients with SLE.     

Primary Sj?gren's syndrome in men. Clinical, serologic, and immunogenetic features

Although primary Sj?gren's syndrome is a common rheumatic disorder in women, it is not well recognized in men. This study represents the first report of the clinical, serologic, and immunogenetic features of a group of 36 men with primary Sj?gren's syndrome, which are contrasted with those of a group of 69 women with primary Sj?gren's syndrome. The majority of male patients had extraglandular involvement including articular (78 percent), neurologic (39 percent), inflammatory vascular (25 percent), and lymphoproliferative disorders (17 percent). Although men were at the same risk for the development of extraglandular complications, there were significant serologic and immunogenetic differences. In sharp contrast to women with Sj?gren's syndrome, men with Sj?gren's syndrome were seronegative with respect to the presence of serum rheumatoid factor (p = 0.008) and antibodies to Ro(SS-A) (p = 0.016). The supertypic specificity, MT2 (DRw52), as in women, was strongly associated with primary Sj?gren's syndrome in men when compared with race-matched control subjects (p = 0.0015). In men, however, the frequency of HLA-B8 and HLA-DR3, the most common DR locus specificity observed in women, was not statistically different from that observed in the normal control group.