Pregnancy in rare autoimmune rheumatic diseases: UCTD, MCTD, myositis, systemic vasculitis and Beçhet disease.

The physiological adaptation of the immune system to pregnancy can potentially affect the course of all autoimmune rheumatic diseases (ARD), conversely the autoimmune processes characteristic of these conditions may compromise the foetal outcome. Unfortunately, very few reports on pregnancy outcome in patients with rare ARD are available. In this paper, we briefly review the data published until now on these disorders. Some general guidelines which were elaborated for more prevalent ARD seem to be valid also for such rare conditions: 1) patients should be correctly informed on the risk of becoming pregnant; 2) pregnancies should be planned when the disease is in remission since it increases the probability of successful maternal and foetal outcome; 3) patients should be regularly monitored during gestation and postpartum by a multidisciplinary team including rheumatologist, obstetrician, and neonatologist; 4) in the case of disease relapse an adequate treatment, even aggressive if necessary, should be recommended since active disease can be more detrimental for foetus than drugs; 5) pregnancies complicated by the onset of rare ARD have a particularly severe prognosis; in these cases a prompt treatment and very close clinical surveillance are indicated.

Health-related quality of life in Italian patients with systemic lupus erythematosus. I. Relationship between physical and mental dimension and impact of age.

OBJECTIVE: To examine health-related quality of life (HRQOL) in Italian patients with systemic lupus erythematosus (SLE) and compare it with that of healthy people, and to investigate relationships among different dimensions and subscales of a generic health status measure. METHODS: The Medical Outcomes Study Short Form-36 (SF-36) was applied in a cohort of 126 consecutive SLE patients and 96 healthy controls. At the time of HRQOL testing, all patients underwent clinical and laboratory evaluation. RESULTS: Both physical (PCS) and mental (MCS) component summary scores of the SF-36 were reduced in patients compared with controls. In SLE great variability in all the subscales was observed. Significant correlations between PCS and MCS and between many different subscales were observed in patients but not in controls. The PCS was higher than MCS more frequently in controls than in SLE patients (81 vs 48.4%, P<0.00001). In SLE, HRQOL tended to worsen with age. CONCLUSION: Both PCS and MCS contribute to the decrease in HRQOL in SLE patients. In SLE the mutual interaction between these two dimensions seems to be more relevant than in healthy people.

Antinucleosome antibodies in SLE: a two-year follow-up study of 101 patients.

We prospectively analyzed the diagnostic sensitivity and specificity as well as the clinical relevance of antinucleosome antibodies in SLE. One hundred and one consecutive SLE patients were followed for 3 years. Three serial serum samples from each patient were tested for antinucleosome antibodies by ELISA (optimum cut-off value 10 U/ml), and for anti-dsDNA antibody (by ELISA and IIF on Crithidia luciliae), and anti-dsDNA avidity (by Scatchard plot analysis). Sera from 100 healthy individuals (HI), 35 patients with systemic sclerosis (SSc), 30 with primary Sjögren's syndrome (SS), 20 with rheumatoid arthritis (RA) and 48 with infectious diseases (ID), were assayed as controls. SLE activity and damage were evaluated using the ECLAM score and the SLICC/ACR index. At baseline, antinucleosome antibodies were found in 87 patients with SLE (86.1%), in 8 patients with SSc (22.8%), in 2 HI (2%), and in 1 ID (2.1%). The sensitivity and specificity of antinucleosome testing for SLE were 86.1% and 95.3%, respectively. The prevalence of antinucleosome antibodies in SLE was significantly higher than that of anti-dsDNA antibodies, with a correlation between them. No relevant relationship was found between antinucleosome antibodies and disease features, including renal involvement, disease activity, and disease damage. During follow-up, no significant variation was observed in antinucleosome level, nor in anti-dsDNA antibody level or avidity. We conclude that antinucleosome antibodies are commonly found in SLE. Low antibody levels can be detected in SSc, whereas medium/high levels are highly specific for SLE. Their clinical relevance during the disease course and utility for monitoring the individual patient seem to be poor.

Risk factors for subclinical atherosclerosis in a prospective cohort of patients with systemic lupus erythematosus.

OBJECTIVE: To evaluate traditional and non-traditional risk factors for subclinical atherosclerosis in systemic lupus erythematosus (SLE). METHODS: A prospective cohort of 78 patients with SLE without overt atherosclerotic disease was studied. SLE clinical and laboratory parameters, disease activity and damage, treatment and traditional risk factors for atherosclerosis were evaluated. At baseline (T1) and after five years' follow up (T2), the serum levels of anti-oxidised palmitoyl arachidonoyl phosphocholine (oxPAPC), anti-heat shock protein 65, and anti-beta(2)-glycoprotein I antibodies and C reactive protein were tested. At T2, intima-media thickness (IMT) was measured using duplex carotid sonography. Thickened intima, plaque, mean IMT (m-IMT), and maximum IMT (M-IMT) were assessed. RESULTS: A thickened intima was seen in 22/78 (28%) patients and plaque in 13/78 (17%). M-IMT and m-IMT were (mean (SD)) 0.77 (0.34) mm and 0.55 (0.15) mm, respectively. Patients with carotid abnormalities were significantly older, had higher blood pressure and total serum cholesterol levels, and had taken a higher prednisone cumulative dosage than those without any lesions. The carotid abnormalities were associated with renal disease and ECLAM >2 at T1, and with azathioprine treatment. In multivariate analysis, age and cumulative prednisone dose were associated with carotid abnormalities; age, hypertension, and anti-oxPAPC at T2 were correlated with higher M-IMT and m-IMT. CONCLUSIONS: In patients with SLE some non-traditional risk factors for atherosclerosis were identified, the most important of which was the cumulative prednisone dose. The role of some traditional risk factors, such as age and hypertension, was also confirmed. The predictive value of the new immunological and inflammatory markers of atherosclerosis seems to be masked by some disease related features.

Pregnancy outcome in 100 women with autoimmune diseases and anti-Ro/SSA antibodies: a prospective controlled study.

Anti-Ro/SSA antibodies are associated with neonatal lupus but are also considered a possible cause for unexplained pregnancy loss and adverse pregnancy outcome. In a large multicentres cohort study we have prospectively followed 100 anti-Ro/SSA positive women (53 systemic lupus erythematosus (SLE)) during their 122 pregnancies and 107 anti-Ro/SSA negative women (58 SLE) (140 pregnancies). Anti-Ro/SSA antibodies were tested by immunoblot and counterimunoelectrophoresis. Mean gestational age at delivery (38 vs 37.9 weeks), prevalence of pregnancy loss (9.9 vs 18.6%), preterm birth (21.3 vs 13.9%), cesarean sections (49.2 vs 53.4%), premature rupture of membranes (4.9 vs 8.1%), preeclampsia (6.6 vs 8%), intrauterine growth retardation (0 vs 2.3%)and newborns small for gestational age (11.5 vs 5.8%) were similar in anti-Ro/SSA positive and negative SLE mothers; findings were similar in non-SLE women. Two cases of congenital heart block were observed out of 100 anti-Ro/SSA positive women. In conclusion, anti-Ro/SSA antibodies are responsible for congenital heart block but do not affect other pregnancy outcomes, both in SLE and in non-SLE women. The general outcome of these pregnancies is now very good, ifprospectively followed by multidisciplinary teams with ample experience in this field.

Lack of multimer organization of von Willebrand factor in an acquired von Willebrand syndrome.

We report a case of acquired von Willebrand syndrome (AVWS) in a 20-year-old-woman with systemic lupus erythematosus, in whom severe bleeding complications followed kidney biopsy. Coagulation studies demonstrated undetectable levels of ristocetin-induced platelet aggregation (RIPA), von Willebrand factor antigen (VWF:Ag) and VWF ristocetin cofactor activity (VWF:RCo), associated with significantly prolonged bleeding time; unlike type 3 von Willebrand disease (VWD), platelet VWF was reduced but not undetectable. The plasma VWF multimer pattern was characterized by the presence of only two bands, one of low molecular weight (MW) running as the protomer of plasma VWF in normals, the other of abnormally high MW without detectable intermediate multimers; this pattern resembles that of VWF present in endothelial cells. A search for an anti-VWF antibody demonstrated the presence of an inhibitor at high titre. This anti-VWF antibody did not interfere in the interaction of VWF with platelet glycoprotein (GP) Ib through the A1 domain, and did not react with the A2 domain of VWF; instead, it seemed to modify the relative representation of high and low MW VWF multimers released by normal human umbilical vein endothelial cells (HUVEC). After Azathioprine and corticosteroid treatment, the anti-VWF antibody disappeared and the patient's haemostatic profile normalized, except for the platelet VWF content which still remained decreased. We suggest that the anti-VWF antibody present in the AVWS described compromised both circulating VWF levels and their multimeric organization, inducing the maintenance of the multimer structure that VWF normally has before or in the early phase after secretion from endothelial cells.

[Detection of serum anti-B/B' UsnRNP antibodies in patients with connective tissue diseases by immunoblotting]. / Determinazione all'immunoblotting degli anticorpi sierici anti-B/B' UsnRNP nei pazienti con connettiviti.

OBJECTIVE: To investigate the reliability of the immunoblot method in the detection of serum immunoreactivity towards the B/B' polypeptides of U small nuclear ribonucleoproteins (UsnRNP) and to assess the significance of these antibodies in connective tissue disease (CTD) patients. METHODS: We tested the sera of 348 patients with CTD (101 SLE, 51 systemic sclerosis, 53 primary Sjögren's syndrome, 27 poly/dermatomyositis, 15 rheumatoid arthritis and 101 overlap CTD), of 31 matched healthy subjects and 13 patients with primary Epstein-Barr virus (EBV) infection with high titre of IgG anti-EBV antibodies. IgG anti-UsnRNP antibodies were determined by immunoblotting on nuclear extract from Raji cells (an EBV-immortalised human B lymphoid cell line) and Jurkat cells (a human T lymphoid cell line). Anti-dsDNA antibodies were detected by indirect immunofluorescence on Crithidia luciliae and anti-ENA by counterimmunoelectrophoresis. Anti-dsDNA activity and avidity were measured in SLE sera by ELISA with Scatchard analysis. Results were statistically analysed by chi-square and Mann-Whitney tests. RESULTS: A high frequency of anti-B/B' antibodies was found in the sera of CTD patients, confined to SLE (54.4%) and overlap CTD with SLE features (55,2%). Anti-B/B' immune reactivity was closely associated with other anti-UsnRNP specificities, gel precipitating anti-nRNP and anti-P antibodies. Nine out of 15 (60%) anti-B/B' positive/anti-ENA negative lupus sera on Raji blots were confirmed to be positive also on Jurkat blots. The sera from patients with EBV infection provided, on Raji blots, completely different band patterns from those obtained with auto-immune sera. CONCLUSIONS. The Sm B/B' proteins are the predominant or, at least, the most frequently targeted antigens of the UsnRNP auto-immune response in SLE and "lupus-like" overlap CTD. Moreover, anti-B/B' is diagnostically specific for CTD with SLE features. Immunoblotting on human B lymphoid cells is a reliable method, in terms of sensitivity and specificity, for the detection of anti-Sm B/B' antibodies.

Risk of congenital complete heart block in newborns of mothers with anti-Ro/SSA antibodies detected by counterimmunoelectrophoresis: a prospective study of 100 women.

OBJECTIVE: To assess the true prevalence of congenital complete heart block (CCHB) in infants of anti-Ro/SSA-positive women known to have connective tissue disease (CTD) and, secondarily, to evaluate the prevalence of other electrocardiographic abnormalities in these newborns at birth. METHODS: A prospective study was conducted in 4 referral hospitals. One hundred anti-Ro/SSAA-positive mothers were followed up before they became pregnant and during the index pregnancy. Counterimmunoelectrophoresis and immunoblotting were used to test for antibodies to extractable nuclear antigens. RESULTS: Of the 100 women with anti-Ro/SSA antibodies, 2 had infants who developed CCHB in utero (2%). The CCHB was detected at 22 weeks and 20 weeks, respectively. One of the 2 mothers had primary Sjögren's syndrome (SS), and the other had undifferentiated CTD (UCTD). No case of CCHB occurred among the infants of 53 mothers with systemic lupus erythematosus (SLE). No fetal death occurred due to CCHB. In 2 centers, electrocardiography was recorded in 24 unselected newborns, and 4 were found to have sinus bradycardia. CONCLUSION: The prevalence of CCHB in newborns of prospectively followed up women already known to be anti-Ro/SSA positive and with known CTD was 2%. This finding is useful with regard to preconception counseling of these women. The risk of delivering an infant with CCHB may be higher in mothers with primary SS or UCTD than in those with SLE. Additional electrocardiographic abnormalities such as sinus bradycardia and prolongation of the QT interval may be present in their children.

[Quality of life in systemic lupus erythematosus] / La qualità della vita nel lupus eritematoso sistemico.

The SF-20 and the SF-36 are the most frequently used questionnaires for assessing the quality of life in SLE patients. The SF-36 is actually considered the most suitable for this disease, due to the inclusion of fatigue, a manifestation frequently observed in SLE patients. Using these instruments, it has been clearly demonstrated that patients with SLE have a worse quality of life than healthy people of the same age. Some aspects of daily life, like physical activity, job, social relationship and vitality, are particularly affected. In the majority of studies, an inverse relation between quality of life and disease activity has been observed. The influence the damage has on the quality of life is more complex, since a greater number of variables are involved. In fact, the amount of damage largely depends on the organ involved and on functional impairment resulting from it. To explain the variability in the quality of life among different patients, it is important to consider, besides the clinical complaints, the psycho-social dimension of each person. In fact, some SLE patients, unlike others, cope well with the disease. People behave differently when faced with critical situations, i.e. after being diagnosed with a chronic disease; their reaction depends on the degree of support they receive from family, friends and colleagues, and from the different strategies of coping, that they use.

[Imaging in major salivary gland diseases] / Diagnostica per immagini nelle malattie delle ghiandole salivari maggiori.

Most of the salivary glands diseases are characterized only by a few distinct clinical patterns. Medical history and clinical examination are still considered of great relevance. However, in order to obtain a definite diagnosis, imaging techniques are required in most of the cases. Salivary glands ultrasonography (US) is the technique to be used as the first because US can easily differentiate calculosis, inflammatory diseases and tumors. Sonography is also frequently needed to perform needle aspiration or biopsy (FNAC). Sialography should be used essentially for assessing chronic sialoadenitis as well as Sjögren's syndrome. At present, Magnetic Resonance sialography should be preferred because of the greater sensibility in diagnosing inflammatory diseases of the salivary glands. It allows to evaluate both intraglandular oedema and nodules, so that incannulation of the salivary duct is not required. Computer Tomography (CT) and Magnetic Resonance imaging (MR) are useful when neoplasm are suspected, particularly if deep areas of the gland, which cannot be visualized by US, are involved. Sequential scintigraphy is currently employed for assessing the functional status of all the 4 major salivary glands and evaluating the chronic evolution of glandular damage.

Anti-ribosomal P protein antibodies detected by immunoblotting in patients with connective tissue diseases: their specificity for SLE and association with IgG anticardiolipin antibodies.

OBJECTIVE: To assess the prevalence and clinical and serological associations of anti-ribosomal P protein antibodies (anti-P antibodies) in patients with connective tissue diseases (CTDs) and investigate the immunobiological nature of autoantibody clustering in which anti-P antibodies play a part. METHODS: IgG anti-P antibodies in the sera of 267 patients with CTDs and 31 healthy subjects were analysed by immunoblotting performed on cytoplasmic extract of Raji cells. 60 patients with systemic lupus erythematosus (SLE), 32 systemic sclerosis, 46 primary Sjögren's syndrome, 16 poly/dermatomyositis, 11 rheumatoid arthritis, 8 undifferentiated CTD, 72 overlap CTD, and 22 primary antiphospholipid syndrome were studied. Anti-P antibodies were affinity purified by elution from nitrocellulose bound antigen and tested by ELISA for their binding activity to cardiolipin. RESULTS: Anti-P antibodies were detected in 16 (6%) patients and in none of the controls: 12/60 SLE (20%) and 4/80 undifferentiated/overlap patients with CTD (5%). A close association of IgG antibodies with P proteins and with cardiolipin was seen in lupus sera (p=0.0009, odds ratio 18.33). Anti-P antibodies from 9 of 12 anti-P lupus serum samples could be affinity purified and none of the affinity purified fractions cross reacted with ELISA plate coated cardiolipin. CONCLUSIONS: Anti-P immunoreactivity is a specific marker of SLE and lupus-like disease and its detection is recommended as a powerful diagnostic tool. Anti-P antibodies are strongly clustered with IgG anticardiolipin antibodies in lupus sera, even if they are independently elicited. This suggests that their cognate autoantigens play a part in a common pathogenetic pathway in SLE.

The use of Tween 20 in immunoblotting assays for the detection of autoantibodies in connective tissue diseases.

Autoantibodies directed against intracellular antigens can be detected by immunoblotting (IB). Due to its high sensitivity this technique has many advantages, but it can give misleading results when the specific bands are weak or blurred against the background staining. To decrease background staining, non-ionic detergents (Tween 20, Triton X-100, Nonidet P-40) are generally used as blocking agents. Moreover, these agents appear to have a renaturating action towards proteins and antigens. Tween 20 has a more pronounced renaturating effect on proteins than other detergents and thereby improves antigen-antibody binding. To evaluate the effect of Tween 20 on specific autoantibody detection by IB, we tested the sera of 162 patients with connective tissue diseases (CTDs) by adding this detergent at certain steps of the IB assay. We found that the use of Tween 20 in the IB procedure significantly improved the binding of autoantibodies to Jo-1, Scl70, (U1)RNP 68 kDa and C, Sm B/B' and D. Moreover, it increased the sensitivity for the detection of anti-Sm D peptide in systemic lupus erythematosus (SLE) sera with no decrease in specificity. In contrast, the addition of Tween 20 significantly decreased the binding of autoantibodies specific for ribosomal P proteins, La/SSB, Ro/SSA, but not the overall sensitivity and specificity of the method. We conclude that the addition of Tween 20 to standard IB is advantageous for anti-nuclear antigen antibody detection and improves the sensitivity of the method in revealing anti-Sm-positive sera in SLE. However, Tween 20 is not recommended for the detection of anti-cytoplasmic antibodies.

Psoriatic arthritis exacerbated by Salmonella infection.

Psoriatic arthritis (PsA) is an inflammatory joint disease in which environmental factors, particularly trauma and infections, are thought to play an important role. The authors describe the case of a patient with a mild and long-untreated form of PsA which was severely exacerbated by Salmonella typhimurium infection. This case confirms the importance of infectious agents in the occurrence and course of PsA.

Clinical and laboratory manifestations of elderly onset psoriatic arthritis: a comparison with younger onset disease.

OBJECTIVE: Although the influence of age on clinical and laboratory features has been widely demonstrated in many arthropathies, studies on elderly onset (> 60 years) psoriatic arthritis (EOPsA) are rare. This study compares manifestations at onset and two year outcome of EOPsA with those of younger onset PsA (YOPsA). PATIENTS AND METHODS: Sixty-six consecutive PsA patients with disease duration < 1 year, 16 EOPsA (> 60 years) and 50 YOPsA (< or = 60 years) were admitted to a prospective study. Clinical, laboratory, and radiographic assessment were carried out at admission and after two years. HLA class I and bone scintigraphy were also recorded. In 10 patients with EOPsA and 24 with YOPsA it was possible to obtain synovial fluid, which was subsequently analysed for local inflammatory indices, including interleukin (IL) 1 beta, IL6, and IL8. RESULTS: Presenting manifestations of EOPsA differed from YOPsA in number of active joints (mean (SD)) (12.2 (6.3) v 6.7 (4.6), p < 0.001), foot bone erosions (2.7 (1.2) v 1.1 (1.1), p < 0.001), erythrocyte sedimentation rate (64.2 (35.3) v 30.5 (30.0) mm 1st h, p < 0.001), C reactive protein (3.9 (2.0) v 1.3 (1.3) mg/dl, p < 0.001) and synovial fluid IL1 beta (8.0 (4.7) v 3.0 (3.0) pg/ml, p < 0.001) and IL6 (828.2 (492.6) v 469.3 (201.4) pg/ml, p < 0.005). No differences were found in the number of subjects with dactylitis, pitting oedema, HLA-B27, or signs of sacroiliac and sternoclavicular joint involvement at bone scintigraphy. After two years, progression was more evident in EOPsA than in YOPsA, as the number of new erosions in the hands and also the C reactive protein were higher in EOPsA patients. CONCLUSION: PsA has a more severe onset and a more destructive outcome in elderly people (onset > 60 years) than in younger subjects. This behaviour may be influenced by immune changes associated with aging, as suggested by the higher concentrations of IL1 beta and IL6 found in the synovial fluid of EOPsA than in YOPsA.

Outcome of fifty-five newborns of antiphospholipid antibody-positive mothers treated with calcium heparin during pregnancy.

OBJECTIVE: The outcome of 55 infants born to 53 antiphospholipid antibody (aPL)-positive mothers treated during pregnancy with calcium heparin is described. METHODS: The clinical state of the children was evaluated immediately after delivery by a clinical examination, and a neonatological check-up was performed no later than 24 hours after birth. Neonates with problems were transferred to the neonatal intensive care unit. After their discharge from hospital the clinical state of the babies was followed by means of interviews with the pediatricians and mothers for a period varying between 1.33 and 5.66 years (mean 2.51 +/- 0.92 SD). RESULTS: The newborns comprised 30 females and 25 males, including 2 sets of twins, delivered between the 25th and 40th weeks of gestation (mean 36.69 +/- 2.91 SD). They had a mean birth weight of 2.828 g +/- 706.50 SD (range 800-4.000) and a mean Apgar score at 5 minutes of 9.60 +/- 0.68 SD (range 7-10). Soon after delivery, 12 children (21.81%) were admitted to the neonatal intensive care unit for periods varying between 2 and 120 days (mean 30.33 +/- 33.40 SD), after which the clinical course was normal. All of these neonates suffered from complications exclusively due to prematurity. Malformations and signs of thrombosis or other aPL-related disorders were not observed in any of the newborns. During the follow-up, none of the diseases suffered by the 55 children differed from those of the normal pediatric population; in particular, aPL-related manifestations were never observed. CONCLUSION: These data indicate the absence of aPL-related problems in the offspring of aPL-positive mothers treated during pregnancy with calcium heparin.

Thyroid gland disorders in primary Sjögren's syndrome.

OBJECTIVES: To evaluate the frequency of thyroid disorders in primary Sjögen's syndrome. PATIENTS AND METHODS: 121 consecutive patients meeting Vitali's criteria for primary Sjögren's syndrome and 74 with rheumatoid arthritis underwent thyroid hormone assays, tests for antimicrosomal and antithyroglobulin antibodies, tests for antinuclear antibodies and antibodies to extractable nuclear antigens. Antimicrosomal and antithyroglobulin antibodies were also assayed in 404 controls. RESULTS: frequencies were calculated separately in males and females, and data in females were subjected to statistical analysis. As compared with controls, Sjögren's syndrome patients were more likely to have antimicrosomal antibodies (9% versus 17.6%, P < 0.05) and both Sjögren's syndrome and rheumatoid arthritis patients were more likely to have antithyroglobulin antibodies (1% versus 13.4% and 10.9%, respectively, P < 0.0001). Hypothyroidism was more common among Sjögren's syndrome patients (13.4%) than rheumatoid arthritis patients (3.1%) (P < 0.05). Sjögren's syndrome patients with thyroid disorders were less likely to have antinuclear antibodies, rheumatoid factors or a Chisholm's stage 3 or 4 lip biopsy. CONCLUSIONS: our data confirm that thyroid disorders are more common in primary Sjögren's syndrome than in rheumatoid arthritis and controls. Production of autoantibodies and severe histologic lesions were less common in Sjögren's syndrome patients with than without thyroid disorders.

Juvenile onset of primary Sjögren's syndrome: report of 10 cases.

OBJECTIVE: Ten new cases with primary Sjögren's syndrome (pSS) whose disease began before age 16 are described. Special attention is paid both to the follow-up and treatment of this condition. METHODS: Cases with juvenile pSS were retrospectively identified from our series of 180 pSS patients. Ocular, salivary, and extraglandular manifestations as well as a full laboratory evaluation including HLA-DR typing were retrieved. RESULTS: A disease prevalence of 5.5% (10 cases, 8 female and 2 male) was found in our series. The mean age at onset was 11.0 years, but the disease started at the age of 4 in 2 patients. At onset, parotid swelling was found in 6 cases and extraglandular manifestations in 3. Throughout the follow-up period (mean 48.6 months from the time of diagnosis), the clinical picture was similar to that of pSS in adults, but oral involvement was generally milder. Extraglandular manifestations were always present but never severe. Pertinent laboratory abnormalities (e.g. rheumatoid factor, polyclonal hypergammaglobulinemia, leukopenia, increased ESR, ANA and anti-SSA/SSB antibodies) were found in all patients. Specifically, ANA and anti-SSA were always positive. Moreover, in our cases histocompatibility antigens HLA-DR3 and DR 52 were closely associated with the disease. Clinical outcome was difficult to predict; however, no serious complications have been observed so far. We obtained good results with low-dose steroids and/or hydroxychloroquine, especially with regard to the extraglandular manifestations and laboratory abnormalities. CONCLUSION: We confirm that juvenile pSS is not a rare condition. It closely resembles pSS in adults except for the extremely high prevalence of recurrent parotitis and immunological findings.