Evaluation of the serum ß2 Microglobulin level in patients with systemic lupus erythematosus and its correlation with disease activity.

BACKGROUND: Designation of disease activity is serious for the management of systemic lupus erythematosus (SLE). Serum level of ß2 microglobulin (ß2M) may be associated with illness activity in SLE disease. Since the role of ß2M for assessing of illness activity in SLE is not completely clear, the current study aimed to discern evaluation of ß2M in patients with SLE and its correlation with sickness activity. MATERIALS AND METHODS: In this case-control study, 50 patients with SLE disease and 25 healthy individuals were selected in Imam Khomeini Hospital in central of Urmia. Blood samples were collected safely from patients, serum was removed, and ß2M measured using an ELISA method. The results for other parameters including C reactive protein, C3, C4, anti dsDNA and erythrocyte sedimentation rate were obtained from patients' medical record. Data analyzed using appropriate statistical tests including Mann-Whitney U test, Independent f-test, Kruskal-Wallis, and Spearman used for analysis of data. RESULTS: In the current study, a significant difference was seen between two groups in terms of ß2M (p < 0.001). Remarkable correlation was seen between the level of ß2M with disease activity (p < 0.001). Furthermore, there are significant relevancy between the level of ß2M with 24-hour urine protein, ESR, disease activity score, and CRP (p < 0.05). CONCLUSION: The results revealed that serum amount of ß2M in SLE patients is higher compared to healthy ones, which is significantly correlated to score of illness activity, CRP, and ESR in patients with SLE disease. Hence ß2M might be an excellent serological marker helping the prediction of sickness activity and inflammation in SLE patients.

Postsplenectomy Kawasaki disease in a 17-year-old male.

Kawasaki disease (KD) is characterized with an acute systemic vasculitis of the medium- and small-sized vessels. This disease mainly involves children within the age of 6 months to 5 years and it is often self-limited and patients seem to recover well; however, it may lead to devastating and fatal cardiovascular complications such as coronary artery aneurysm. Thus, early diagnosis and appropriate management of this disease have a significant effect on improving the prognosis and preventing its serious complications. Adult-onset KD (AKD) is rare and often misdiagnosed. Here we report a rare case of KD that occurred in a 17-year-old young adult who presented 4 weeks post splenectomy, with clinical signs and symptoms consistent with AKD. This may potentially highlight the association of AKD with infectious etiologies.

Evaluation of serum uric acid level in systemic lupus erythematosus patients with normal and high pulmonary arterial hypertension.

BACKGROUND: Systemic lupus erythematosus (SLE) is a life-threatening multisystem inflammatory condition affected any organ system. Considering the role of uric acid as a pro-inflammatory compound in high pulmonary arterial pressure, serum levels of uric acid and its relation to severity and duration of the disease were assessed in SLE patients. METHODS: As a cross-sectional study, 75 patients with SLE were enrolled in Imam Khomeini Hospital and Sahand Clinic. Serum uric acid level was measured by pars azmoon kit. Pearson correlation coefficient and T-test were used for statistical analysis of data. RESULTS: The mean duration of SLE was 56.44 ± 40.57 months. High serum uric acid was observed in 13.3% of patients with SLE. Moreover, 8% of these patients had high pulmonary arterial pressure. Serum uric acid in patients with high pulmonary artery pressure was significantly higher than patients with normal pulmonary artery pressure (P < 0.01). Furthermore, a significant relation was seen between severity of SLE disease with serum uric acid level and pulmonary artery systolic pressure (P < 0.05). However, there was no significant correlation between serum uric acid level and duration of the disease (p = 0.90, r = 0.016). CONCLUSION: According to these results, a significantly increased level of serum uric acid was observed in patients with pulmonary arterial pressure. Hence, serum uric acid level could be a prognostic marker of pulmonary arterial pressure in SLE patients which correlates with disease severity. It also would help to reduce clinical demands for echocardiography in patients with normal uric acid levels.

Very Rare Presentation of Cerebrovascular Accident in 20-Year-Old Man With Familial Mediterranean Fever-Case Report.

Familial Mediterranean fever (FMF) is characterized by recurrent episodes of fever accompanied by serosal, synovial, or cutaneous inflammation. The central nervous system (CNS) is rarely involved in FMF. The CNS involvement includes demyelinating lesions, posterior reversible encephalopathy syndrome, pseudotumor cerebri, optic neuritis, and cerebral vasculitis. Here, we present a 20-year-old man, a known case of FMF with abrupt left-sided hemiparesis. Brain magnetic resonance imaging revealed right periventricular infarction. Normal echocardiography ruled out cardioembolism, and thrombophilia workup was negative. Therefore, FMF-induced cerebrovascular accident was considered. Although rare, CNS involvement as a result of FMF disease should also be considered when encountering patients with FMF and CNS manifestations.

Diagnostic performances of high resolution trans-thoracic lung ultrasonography in pulmonary alveoli-interstitial involvement of rheumatoid lung disease.

INTRODUCTION: Alveolointerstitial involvement is a common manifestation in patients with connective tissue disorders. The aim of our study is to investigate the utility of modified TTUS scoring system compared with HRCT findings of pulmonary involvement in rheumatoid lung disease. MATERIAL AND METHODS: Thirty one consecutive patients with a suspected diagnosis of rheumatoid lung involvement were examined with high resolution transthoracic ultrasonography for detecting of ultrasonographic comet tail signe as a ultrasonographic marker of lung involvement in alveolointerstitial involvement in rheumatoid lung disease and the results of them were compared with High resolution computed tomography as gold standard method for diagnosis of lung involvement in this patients. RESULTS: In comparison with HRCT as gold standard method, the sensitivity, specificity, positive and negative predictive value of TTUS was 73.58%, 88.23%, 95.12% and 51.72% respectively. CONCLUSION: Modified TTUS can be a useful imaging modality in the evaluation of even early stages of pulmonary involvement in rheumatoid lung disease.

Evaluation of disease activity in ankylosing spondylitis; diagnostic value of color Doppler ultrasonography.

INTRODUCTION: Spondyloarthropathies including ankylosing spondylitis (AS) require early diagnosis to prevent irreversible changes. Sacroiliitis is a common initial manifestation of AS and is frequently diagnosed by magnetic resonance imaging (MRI). The goal of our study was to assess color Doppler ultrasonography as a potential diagnostic tool in suspected sacroiliitis in comparison with MRI representing the gold standard. MATERIALS AND METHODS: Fifty-one consecutive patients with AS and sacroiliitis and 30 control subjects underwent contrast-enhanced MRI and high resolution color Doppler and duplex ultrasonography of both sacroiliac joints (SIJ) for the detection of vascularization and blood flow spectral Doppler waveform analysis. RESULTS: MRI demonstrated active disease in 27 and inactive disease in 24 patients. CDUS detected pulsatile monophasic wave spectral waveform flow in 22 patients with the active disease, and triphasic in 7 patients with inactive disease and in 8 control patients. The sensitivity, specificity, positive predictive value and negative predictive value for active sacroiliitis detection with CDUS were 82 % (95 % CI, 68-91 %), 92 % (95 % CI, 85-96 %), 91 % (95 % CI, 84-96 %), and 84 % (95 % CI, 70-92 %), respectively, for pulsatile monophasic wave spectral waveform Doppler sonography. MRI of SIJ was negative in all 30 (60 SIJ) control participants. CONCLUSIONS: Our results show that CDUS is a practical and useful tool in the diagnosis of active sacroiliitis.

Serum levels of IL-18 in Iranian females with systemic lupus erythematosus.

UNLABELLED: IL-18 is a member of the IL-1 family of cytokines but due to its unique inflammatory and imimunoregulatory properties it is suggested that it plays important roles in autoimmune and inflammatory diseases like as systemic lupus erythematosus (SLE). METHODS: The current study was sought to evaluate serum levels of IL-18 in Iranian females with SLE. Serum samples of 25 patients with low disease activity, SLE-DAI score < 7, 25 with high disease activity, SLE-DAI score > or = 7 and 25 normal subjects were assessed for IL-18, anti-ds-DNA, C3, C4 and other lab findings using appropriate methods. Level IL-18 in patients with SLE-DAI score > or = 7 was significantly higher than patients with low disease activity and controls (p = 0.026 and p = 0.005, respectively). RESULTS AND DISCUSSION: In patients with high disease activity, a positive correlation was found between serum levels of IL-18 with DsDNA antibody (r2 = 0.38, p = 0.03), protein levels in 24 hours collected urine (r2 = 0.53, p = 0.007), platelet counts (r2 = 0.506, p = 0.01) and it correlates negatively with serum C3 levels (r2 = -0.42, p = 0.02). CONCLUSION: The findings point to important role of IL-18 in SLE patients with disease activity higher than SLE-DAI score 7.