Shrinking lung syndrome in systemic lupus erythematosus patients with dyspnea

To identify the frequency of shrinking lung syndrome [SLS] in systemic lupus erythematosus [SLE] with dyspnea and study the clinical characteristics and differences in disease activity and damage. The study included 47 SLE patients complaining of dyspnea. SLS was considered in those with exertional dyspnea, restrictive pulmonary function tests [PFTs] and elevated copula of the diaphragm. Full history taking, thorough clinical examination, laboratory and relevant radiological investigations were performed for all the patients. Systemic Lupus Erythematosus Disease Activity Index [SLEDAI] and Systemic Lupus International Collaborating Clinics [SLICC] indices were compared. High resolution CT chest was performed for patients with radiological findings consistent with SLS. The mean age of the patients was 29.43 +/- 7.45 years, mean disease duration 5.18 +/- 3.62 years. The SLS was present in 8 patients [17.02%]. There was bilateral elevation of the diaphragm copulae in 25% of SLS patients and two had associated basal atelectatic bands. The serum uric acid was significantly higher in those with SLS while the 24 h urine protein was significantly lower and C4 normalized. The levels of SLEDAI and SLICC tended to be lower in those with SLS, yet there was no significant difference from those without. The demographic features, clinical and laboratory manifestations, disease activity and damage scores, PFTs and radiological findings of the SLE patients are presented. In SLE patients with dyspnea, SLS should be looked for as it is present in a high proportion of cases

Juvenile and adult onset systemic lupus erythematosus outcome in Egyptian patients

The aim was to study the outcome characteristics of systemic lupus erythematosus [SLE] in Egyptians according to the age at disease onset and gender. We studied 239 SLE patients [185 adult and 54 Juvenile onset] with a female to male ratio of 9.39-1 and a mean age of 28.23 +/- 8.91 years and disease duration of 5.45 +/- 4.25 years. Full history taking, thorough clinical examination, laboratory and relevant radiological investigations were performed. Disease activity was assessed using SLEDAI and damage by SLICC. Renal biopsies were done in those with renal involvement. The clinical manifestations, disease activity and damage and laboratory investigations of the SLE patients varied according to the age at disease onset and gender. The prevalence of damage was obviously increased in juvenile patients and higher in males. Growth failure, delayed puberty and fibromyalgia were present more in Juvenile-onset patients. Adult onset SLE patients had a significantly higher secondary Sjogren syndrome especially in females. In the present study, there was a 2.5% mortality and the commonly involved kidneys were an important cause of death. Measuring organ damage in SLE is important with special concern to juvenile-onset patients to allow for designing new treatments that improve control of disease activity and minimize the development of irreversible damage. The kidney appeared to be commonly involved, especially in males, indicating the importance of regular screening for early and appropriate management

Sjogren syndrome and fibromyalgia after radioiodine therapy in cancer thyroid patients

Salivary and lacrimal gland dysfunction is relatively frequent after radioiodine therapy. An association of Sjogren's syndrome [SS] and other autoimmune rheumatic diseases as fibromyalgia syndrome [FMS] has been reported. Thyroid autoimmunity in FMS patients is higher than normal subjects. To detect the occurrence of Sjogren syndrome [SS] and any rheumatologic association in cancer thyroid patients after radioactive iodine therapy [I -131] and evaluate the salivary and lacrimal glands function. Thirty-one patients with post-surgical differentiated thyroid carcinomas with a mean age 40.13 +/- 9.82 years, were referred for I-131 therapy [mean dose 212.9 +/- 101.63 mCi] and continued the follow-up study. All patients had no symptoms or signs of SS. Thorough rheumatological examination was performed for any musculoskeletal manifestation or associated fibromyalgia syndrome [FMS]. Before and 8-12 months after I-131 therapy, salivary glands function was estimated by sequential scintigraphy, while lacrimal gland function was assessed by Schirmer's test. Antinuclear antibody [ANA], anti-Ro [SS-A], anti-La [SS-B] and rheumatoid factor [RF] were performed. All patients had a normal salivary glands scintigraphy and Schirmer's test before I-131 therapy. On follow up, primary SS occurred in 8 patients [25.81%] while a significant decrease in salivary function occurred in 18 [58.1%] patients and significantly correlated with the I-131 dose. Schirmer's test was significantly abnormal in those with SS. Serum Anti Ro and Anti La levels became significantly higher in SS patients [18.25 +/- 11.61 and 25 +/- 13.06 U/ml] compared to the others [6.57 +/- 1.8 and 7.35 +/- 1.8 U/ml], respectively, [p 0.025 and 0.006]. Fibromyalgia syndrome was present in 12 patients [38.71%] and 6 of them developed SS. Assessment and follow up of salivary and lacrimal glands function is essential in patients receiving radioiodine therapy. Abnormal level of anti-Ro and Anti-La increase the risk for SS that should be closely monitored and fibromyalgia is a common association

Serum COMP and their correlations with various disease parameters in patients with systemic lupus erythematosus and osteoarthritis

The cartilage oligomeric matrix protein [COMP] is a glycoprotein, which occurs mainly in an articular cartilage. The amount of this protein increases under the influence of cytokines and growth factors. As a result of various diseases that cause damage to cartilage, fragments of matrix protein are released into synovial fluid and then into blood. The assessment of matrix protein level in serum, for example COMP, permits the establishment of the degree of cartilage damage in inflammatory joint diseases, and permits observation of the effectiveness of the treatment. To assess serum COMP level, as a marker for cartilage degradation, in SLE and OA patients and to find a correlation between serum COMP level and other markers as well as activity of disease, disease duration and the age of the patients. Blood was collected from 40 systemic lupus erythematosus [SLE] patients group I, [the patients were further subdivided into two subgroups, group [Ia] comprised 20 SLE patients received 1 g IV methylprednisolone [MP] daily for three successive days, group [Ib] comprised 20 SLE patients did not receive IV methylprednisolone [MP]], and from 20 patients with knee osteoarthritis [OA] group II who constituted the control group. Serum COMP level was determined using an inhibition enzyme-linked immunosorbent assay [ELISA]. The measured values of the serum COMP level in SLE patients ranged from 1.32 to 1.71 microg/ml with a mean of 1.51 +/- 0.13 microg/ml in group [Ia], and ranged from 2.43 to 3.56 microg/ml with a mean of 2.86 +/- 0.31 microg/ml in group [Ib]. While in OA group [II] the value of serum COMP ranged from 0.97 to 2.65 microg/ml with a mean of 1.25 +/- 0.37 microg/ml. We found significantly elevated COMP levels in the SLE group [Ib] compared to the SLE group [Ia] patients and OA group [II] [p < 0.001]. We found a statistically significant positive correlations with the number of tender joints [correlation coefficient Pearson's: r = 0.45, p < 0.01], the number of swollen joints [r = 0.55, p < 0.001], SLAM value [r = 0.56, p < 0.001]. A significant positive correlation was found between serum COMP level and the ESR value in the first hour [r = 0.35, p < 0.001]. While the serum COMP level was independent of the patients' age [r = 0.04, p = NS], disease duration [r = -0.03, p = NS] and morning stiffness duration [r = -0.05, p = NS]. Also a Negative correlation was found between the serum COMP level and haemoglobin value [r = -0.11, p = NS]. As regards the OA group, no correlation was found between the serum COMP level and patients' age [r = -0.05, p = NS] and disease duration [r = 0.24, p = NS]. There were positive correlations between serum COMP and WOMAC index score for the lower limbs [r = 0.64, p < 0.05]. The serum COMP level can be an important marker of disease activity and cartilage destruction in SLE and OA Patients, and that serum levels of COMP can be used as a parameter for monitoring the therapy response in SLE patients undergoing an intravenous bolus steroid therapy

Serum BAFF level and its correlations with various disease parameters in patients with systemic sclerosis and systemic lupus erythematosus

Interest in B-cells has been revived due to the description of new functions. Supporting a role for B-cells in the genesis of autoimmune diseases is the fact that the B-cell-activating factor of the TNF ligand family [BAFF] is essential in their physiology. The role of BAFF, a new cytokine, in autoimmune diseases has been highlighted. To assess serum BAFF level in systemic sclerosis [SSc] and systemic lupus erythematosus [SLE] to verify its role in these diseases and find any relation with the clinical manifestations, laboratory investigations, disease activity and damage. The study included 12 SSc and 40 SLE patients. The patients were subjected to full history taking and thorough clinical rheumatological and dermatological examinations and relevant investigations including autoantibodies and CT chest in SSc. In SSc, the total skin thickness score was scored according to the modified Rodnan skin score [MRSS] method. In SLE, the disease activity was assessed using the Systemic Lupus Activity Measure [SLAM] and organ damage using the Systemic Lupus International Collaborating Clinics/ACR [SLICC/ACR] index. The serum BAFF levels were measured using a specific ELISA. The BAFF level was remarkably elevated in SSc and SLE in a comparable percentage of patients, yet the level was highest in SLE and lower in the limited SSc subtype. The BAFF significantly correlated with the level with the MRSS in SSc and with both the SLAM and SLICC in SLE patients. The elevated level of BAFF in SSc further confirms the importance for new therapeutic targets for its inhibition to slow the disease progression, particularly skin fibrosis. The role of BAFF in the pathogenesis and disease activity in SLE is well-known and the novel noticeable correlation with the damage index high lightens on the utility of BAFF as an indicator of disease damage and predictor of poor outcome