2.20 Behcet’s disease and miscellaneous rheumatic conditions

Background: Behcet’s disease is an inflammatory, systemic and chronic disorder with unknown etiology affecting multiple systems of body (1). The cause is not clear but seems to be multifactorial, including immune system dysfunction (humoral and cellular immune defects), endothelial cell dysfunction and genetic predisposition (2). White adipose tissue produces variety of proteins in the name of adipocytokines, with important roles in body metabolism. One of these newly identified secreted adipocytokines is visfatin, which is secreted by the visceral fat and its plasma level increases during the obesity. It has insulinmimetic effects in metabolism of cultured cells and activates the insulin receptor (3). Visfatin stimulates inflammatory cells like monocytes and can induces increasing circulating level of IL-6 in mice. It have been considered as a new proinflammatory adipocytokine (4). Previous studies have evaluated visfatin level in immunologic disorders like rheumatoid arthritis and showed it was significantly higher in comparing to control subjects (4,5,6). There was no evaluation in patients with behcet disease yet. Objectives: We have evaluated visfatin level in patients with behcet disease finding inflammatory role of that in pathogenesis and clinical manifestations of behcet disease. Methods: We have evaluated 40 patients with Behcet’s disease fulfilled the International Study Group Criteria for the Diagnosis of Behc¸et’s Disease (ISG) and 40 healthy subjects from healthy candidates referring to behcet clinic of Shiraz medical university as a referral center for these patients in south Iran. Both groups have been matched for age, body mass index (BMI) and sex. Visfatin was checked in both groups using ELISA Kit. Results: There were no significant difference between cases and controls in mean concentration of visfatin level (P = 0.61). Difference in the visfatin level between patients with active and inactive manifestations of Behcet’s disease approximated to the significant levels (6.13 3.20 and 4.25 2.73, respectively; P = 0.07). Conclusion: In view of our study, we have concluded that visfatin levels may affect the clinical manifestations of BD maybe as a proinfalmmatory marker in pathogenesis and active manifestations of Behcet’s disease although more cases should be included in future works

Determination of serum visfatin level in patients with Behcet disease, comparing with normal population

Background: Behcet’s disease is an inflammatory, systemic and chronic disorder with unknown etiology affecting multiple systems of body (1). The cause is not clear but seems to be multifactorial, including immune system dysfunction (humoral and cellular immune defects), endothelial cell dysfunction and genetic predisposition (2). White adipose tissue produces variety of proteins in the name of adipocytokines, with important roles in body metabolism. One of these newly identified secreted adipocytokines is visfatin, which is secreted by the visceral fat and its plasma level increases during the obesity. It has insulinmimetic effects in metabolism of cultured cells and activates the insulin receptor (3). Visfatin stimulates inflammatory cells like monocytes and can induces increasing circulating level of IL-6 in mice. It have been considered as a new proinflammatory adipocytokine (4). Previous studies have evaluated visfatin level in immunologic disorders like rheumatoid arthritis and showed it was significantly higher in comparing to control subjects (4,5,6). There was no evaluation in patients with behcet disease yet. Objectives: We have evaluated visfatin level in patients with behcet disease finding inflammatory role of that in pathogenesis and clinical manifestations of behcet disease. Methods: We have evaluated 40 patients with Behcet’s disease fulfilled the International Study Group Criteria for the Diagnosis of Behc¸et’s Disease (ISG) and 40 healthy subjects from healthy candidates referring to behcet clinic of Shiraz medical university as a referral center for these patients in south Iran. Both groups have been matched for age, body mass index (BMI) and sex. Visfatin was checked in both groups using ELISA Kit. Results: There were no significant difference between cases and controls in mean concentration of visfatin level (P = 0.61). Difference in the visfatin level between patients with active and inactive manifestations of Behcet’s disease approximated to the significant levels (6.13 3.20 and 4.25 2.73, respectively; P = 0.07). Conclusion: In view of our study, we have concluded that visfatin levels may affect the clinical manifestations of BD maybe as a proinfalmmatory marker in pathogenesis and active manifestations of Behcet’s disease although more cases should be included in future works

Fibroblast growth factor-23 in patients with systemic sclerosis: A case–control study

AbstractBackgroundFibroblast growth factor-23 (FGF-23) is actively involved in phosphate homeostasis and skeletogenesis.Aim of the workTo assess the serum level of FGF-23 in systemic sclerosis (SSc) patients (both diffuse dSSc and limited lSSc subtypes) in order to find if it has a role in the pathogenesis of the disease and study its relation to the clinical manifestations.Patients and methodsThe study included 30 dSSc patients, 30 lSSc and 28 age and sex matched controls. In patients, clinical examination and laboratory investigations were performed and Medsger severity scale assessed. Serum FGF-23 was measured using ELISA.ResultsThe age of dSSc patients was 36.94±9.89years and the lSSc 38.36±10.04years. The serum FGF-23 level was 23.44±14.86pg/ml in dSSc patients, 20.01±13.92pg/ml in lSSc patients and 23.09±11.45pg/ml in the control (p=0.58). No significant difference in the FGF-23 level was found according to the presence of lung fibrosis (p=0.6). There was no significant difference in FGF levels among patients according to the severity (p=0.39). In SSc patients there was a significant correlation between FGF and serum phosphorus especially in dSSc patients (r=0.6, p=0.003). Serum urea significantly correlated with FGF-23 in those with dSSc (r=0.46, p=0.037). There was no significant difference in the FGF-23 levels (p=0.18) between those with a normal and impaired glomerular filtration rate.ConclusionThe mean serum level of FGF-23 in this study showed no significant difference between systemic sclerosis patients and their subtypes with the normal subjects. It seems to have no role in the clinical manifestations of the disease

Three atypical manifestations of granulomatosis with polyangiitis: lateral medullary syndrome, anterior cheek mass and melting scleritis of eye

Granulomatosis with polyangiitis (GPA, formerly Wegener granulomatosis) is a vasculitis with various organ involvement. There have been a few cases of CNS stroke and rare cases of lateral medullary infarction (LMI) as a manifestation of GPA. Also there have been reports of sinuses, nose and laryngeal masses mistakenly referred as carcinomas and subsequently GPA was diagnosed in their pathological reports. Another severe fulminant manifestation can be necrotizing scleritis leading to perforation of sclera. Therefore, here we present some rare and fulminant manifestations of GPA in 3 separate cases for further emphasis of the unusual manifestations of GPA that should always be kept in mind

Assessment of hospitalization and mortality of scleroderma in-patients: a thirteen-year study

Objective: Systemic sclerosis (SSc) is an uncommon non-hereditary sporadic disease that increases the risk of premature death, especially in diffuse type. We determined the prevalence of SSc in the last 13 years in our rheumatologic hospitals as a referral center for southern Iranian patients, the causes of hospitalization, the average length of stay (LOS), the mortality rate, and the reason for their mortality. Material and methods : A cross-sectional study was performed in Shiraz University of Medical Sciences, Iran. The studied population included all patients diagnosed with systemic sclerosis. We calculated the hospitalization rates, in-hospital mortality rates, and mean LOS. Results: There were 446 admissions by 181 patients with SSc. The female to male ratio was about 10.7 : 1. The overall mean LOS was 5.95 days. Digital ulcer and interstitial lung disease (ILD) were the most common causes of hospitalizations among the SSc-related events. For those with a non-SSc-related condition, infection was the most prevalent event. Most of the deaths were due to ILD and pulmonary artery hypertension(PAH), and the overall in-hospital mortality rate was 16.5%. Conclusions : Women with SSc had higher rates of hospitalization but lower in-hospital mortality than men.There were some differences between our study and other similar studies in the causes of hospitalization and in-hospital death among SSc patients, especially the lower age of death. The patients with digital ulcers and those with intestinal lung disease or pulmonary hipertension were most commonly admitted to the hospital in our study group. Probably, increasing the skin care of these patients and asking other specialty groups to cooperate will decrease the high rate of hospitalizations in our population

Glomerulopathy in patients with dermatomyositis in early active disease: clinical, pathological and capillaroscopic manifestations, and response to treatment

Introduction Idiopathic inflammatory myopathies (IIMs) are a group of systemic connective tissue diseases that present with muscular and extra-muscular manifestations. There are few reports on kidney involvement, especially in dermatomyositis (DM) patients. We evaluated the clinical, laboratory, capillaroscopy, and kidney pathology of patients with DM, who presented with proteinuria during the first year, and followed them for response to treatment. Material and methods We evaluated 205 patients with proximal muscle weakness or high muscle enzymes, who referred to the nailfold capillaroscopy clinic from April 2010 to October 2021. Seventy-four patients fulfilled the New 2017 EULAR/ACR Classification Criteria for adult and juvenile IM with probability of ≥ 90% for DM with duration of ≤ 12 months and proteinuria > 350 mg/24 hours. All manifestations of patients with glomerulopathy and their kidney biopsies were reviewed, and they were followed for their treatment response. Results From 74 patients with DM, 52 female and 22 male, median age 37 (19–65) years, and disease duration of median 4.5 (1–12) months, 2 (2.7%) patients (25- and 28-year-old male) had proteinuria. Their kidney biopsy showed mesangioproliferative glomerulonephritis (GN). There was no case of acute or chronic kidney damage or rhabdomyolysis. Both had high disease activity, high erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), abnormal capillaroscopy, and high anti-Ro positivity with good early response of their kidney function, muscle weakness, and laboratory tests after immunosuppressive treatment for 3–6 months. One patient had capillaroscopy follow-up, and all abnormalities were resolved in 8 fingers. One patient, due to poor follow-up, after 8 months had recurrence of his disease. Conclusions We found mesangioproliferative GN as a rare extra-muscular manifestation in patients with DM in the active and early phase of the disease. Full immunosuppressive treatment showed early complete recovery in these patients

Huge femoral artery pseudoaneurysm in a patient with Behçet’s disease

Background: Behçet’s disease (BD) is a variable vessel vasculitis and vascular involvement is one of its life threatening manifestations. Arterial involvement frequently occurs with male predominance with pseudoaneurysms being the most common presentation. Immunosuppressive therapy is the mainstay of treatment in vascular involvement. Case presentation: The case we report here is a 40 year old Iraqi BD patient with manifestations of recurrent oral and genital ulcers, bilateral anterior uveitis, and deep vein thrombosis. The pathergy test was positive. The HLA-B51 was negative, erythrocyte sedimentation rate 102 mm/1st h and C-reactive protein was 48 mg/L. After discontinuation of his medications for about 9 months, the disease presented with leg pain and swelling that was diagnosed as huge left superficial femoral artery pseudoaneurysm by Doppler ultrasonography. CT angiography revealed a 90 × 88 × 70 mm pseudoaneurysm with partial mural thrombosis. He was scheduled for emergency surgery due to severe intractable pain. he received a pulse of methylprednisolone 1 g/day for 3 days and then surgery was done in the form of exclusion, repair and femorofemoral bypass were done. Post-operatively, the patient had an uneventful course; distal pulses became palpable, pain and swelling subsided. Post-operation, prednisolone 1 mg/kg was continued and he received cyclophosphamide 750 mg intravenously. His blood homocysteine level was higher than normal 23.8 μmol/L. He was discharged with a high dose of steroid and monthly cyclophosphamide treatment. Conclusion: Arterial pseudoaneurysm is life-threatening in BD and should be kept in mind to prevent major complications. Vascular involvement in BD patients is probably associated with hyperhomocysteinemia

Assessment of inpatients with idiopathic inflammatory myopathies: A 10-year single unit experience

Background: The burden of the patients with idiopathic inflammatory myopathies (IIM) including their admissions, diagnostic and therapeutic cares is similar to other severe diseases. Aim of the work: To evaluate the different aspects of the disease in IIM patients in our region. Patients and methods: All of patients with a diagnosis of IIM admitted to the wards in Shiraz University of Medical Sciences between 2001 and 2011 were evaluated. Results: There were 117 (70.5%) female and 49 (29.5%) male patients. The mean age of the patients was 38.5 ± 16.8 years (range: 4–78 years). Among all admissions, proximal muscle weakness was the most frequent presenting symptom, however, 23 (13.9%) of patients did not report any limb weaknesses. The present study showed evidence of involvement of different systems including, pulmonary, gastrointestinal and cardiac. 35 (21.1%) patients came with polyarthralgia, 58 (34.9%) with dysphagia and regurgitation, and 8 (4.8%) with arthritis. Unexplained fever was noted in 33 (19.9%), myalgia in 25 (15%) and cough and dyspnea in 28 (16.8%) patients. Elevated serum creatine kinase and lactate dehydrogenase were detected in 105 (63.25%) and 153 (92.17%) patients respectively. Electromyogram findings were seen in 113 (91.8%). 35% of the patients had recurrent admissions and mean duration of their admissions was 10 days. During the study period, 8 (4.8%) patients died, mostly with respiratory and then cardiac and infectious complications. Conclusion: IIMs cause various complications and morbidities, with recurrent admissions due to disease flare-up and respiratory and infectious complications. Further prospective studies are recommended to elucidate the predisposing risk factors. Keywords: Hospital admission, Idiopathic inflammatory myopathy, Dermatomyositis, Polymyositis, Mortalit

Serum vascular endothelial growth factor (VEGF), soluble VEGF receptor-1 (sVEGFR-1) and sVEGFR-2 in systemic sclerosis patients: Relation to clinical manifestations and capillaroscopy findings

Introduction: The role of angiogenesis in the pathogenesis of systemic sclerosis (SSc) is well known. The imbalance between vascular endothelial growth factor (VEGF) and their anti-angiogenic soluble receptors (sVEGFR-1 and VEGFR-2) has been proposed as a possible cause of microangiopathy. Aim of the work: To determine the levels of VEGF, sVEGFR-1 and sVEGFR-2 and the VEGF/sVEGFR1 and VEGF/sVEGFR2 ratios in SSc patients and to study their relation with clinical manifestations and capillaroscopy findings. Patients and methods: The study included 44 SSc patients and 44 controls. The sclerosis severity was assessed by the modified Rodnan skin score (mRss) and capillaroscopy performed in patients. Serum VEGF, sVEGFR-1 and sVEGFR-2 were measured in patients and control. Results: SSc patients had a mean age of 40.7 ± 12.8 years, M:F (1:9) and disease duration was 56.2 ± 60.6 months. 27 patients (61.4%) had diffuse-SSc and 17 (38.6%) limited. The mean VEGF was significantly higher (363.4 ± 133.9 pg/ml) and sVEGFR-2 lower (2039.6 ± 109 pg/ml) in patients compared to control (93.9 ± 25.2 pg/ml and 2366 ± 116.5 pg/ml; p = 0.05 and p = 0.04, respectively). Serum levels of sVEGFR-2 in patients with early, active and nonspecific scleroderma pattern of capillaroscopy was higher in comparison to patients with late scleroderma pattern (p = 0.05). There were no significant differences in the studied parameters between those patients with and without digital ulcerations and interstitial pulmonary fibrosis. A significant correlation was found between mRss and VEGF (p = 0.04). Conclusion: An overproduction of VEGF, a potent angiogenic molecule or down regulated production of its natural inhibitors (sVEGFR-2) might be involved in the development of vasculopathy in SSc patients