Current Rheumatology Reviews - Volume 14, Issue 3, 2018

The efficacy of subcutaneous immunoglobulin is reported in several neurological disorders and, more recently, its use has been extended to other inflammatory diseases, such as the idiopathic inflammatory myopathies, including polymyositis and dermatomyositis. Due to the rarity of these disorders, the role of immunoglobulin, administered intravenously or subcutaneously, remains unclear and poorly investigated. We report our experience about the use of subcutaneous immunoglobulin in myositis spectrum disorders, from idiopathic inflammatory myopathies to more complex conditions, such as overlap and cancer-associated myositis or pregnancy.

Background: Rheumatoid Arthritis (RA) is an inflammatory disease with destructive pattern. Patients are suffering from pain and decreased functional outcome as the disease progress. Certain joints are widely discussed in the literature as well as shoulder girdle, but shoulder girdle surgical treatment options' indications and superiorities to each other were not compared entirely. Materials and Methods: Treatment options, such as; synovectomy and bursectomy, resection interposition arthroplasty, hemiarthroplasty, humeral resurfacing arthroplasty, anatomical total shoulder arthroplasty and reverse shoulder arthroplasty, are examined for their timing, advantages, disadvantages and comparison. Results: Patients' age is the main factor about the decision making in rheumatoid arthritis. Young aged patients demand high activity level, but as a result loosening of the implant is frequently encountered. Thus, the protection of the bone stock as much as possible must be the priority. For a young patient with disabling pain should be evaluated for less invasive surgeries such as; synovectomy and bursectomy, resection interposition arthroplasty and also for hemiarthroplasty for preservation of glenoid bone stock. But rotator cuff status, glenoid bone stock evaluation and grading of the glenoid defect become more important in old aged patients and the correct decision making can only be made by combining these factors. Conclusions: Age, functional demand, rotator cuff status and adequacy of glenoid bone stock are defined as major criteria for an optimal treatment. Even though RA patients require additional care for a good functional outcome; with correct decision-making, high quality of life is achievable.

Neuropsychiatric (NP) syndromes are an important cause of morbi-mortality in patients with Systemic Lupus Erythematosus (SLE). Despite remarkable recent progress, there are no definite methods to diagnose neuropsychiatric SLE (NPSLE) or to differentiate primary NPSLE from secondary causes. NPSLE remains a diagnosis of exclusion, and the clinical judgment is still the main approach to the correct diagnosis. Within this complex context, the Magnetic Resonance Imaging (MRI) has both a diagnostic role, by showing the nervous system involvement on one hand and excluding other causes on the other hand, and a prognostic role, help in assessing the lesions and monitoring the evolution. Conventional MRI shows brain involvement in around half of the patients, although the described findings are rather non-specific and present in many other diseases. However, many advanced MRI techniques are becoming increasingly used over the last period, showing abnormalities even in normal-appearing brains on conventional MRI. These MRI techniques hold promise to have a higher sensitivity and specificity than conventional MRI for NPSLE related changes. This review explores the place and the future perspectives of different advanced MRI techniques in NPSLE.

Background: Juvenile scleroderma is a rarely seen chronic connective tissue disorder characterized by stiffening of the skin. The frequency of the disease was reported as one per million. According to organ involvement, the disease is divided into two main forms: systemic and localized scleroderma. Objective: Since it is uncommon in children, many aspects of the disease remain discussable. With this review, we aimed to revise recent findings and new developments in this rare condition. Method: A systematic literature research was performed, using the following medical databases: Pubmed/ Medline and the Cochrane Library. We searched for up-to-date randomized controlled studies, case-control studies, and cohort studies and cases reports on juvenile scleroderma (both systemic and localized form). Results: Skin manifestations are most prominent features of the systemic form, followed by musculoskeletal and vascular involvement. Cardiovascular, gastrointestinal and renal disorders are rare in childhood. Combination of disease modifying anti- rheumatic drugs (methotrexate, mycophenolatemofetil, cyclosporine) and steroid reprents the first line therapy. Bosentan is used for cases with pulmonary hypertension and for extensive digital ulcerations. Biological treatment emerges as a useful treatment option in most severe form of the disease. Localized scleroderma is characterized with sclerodermatosis of the skin. Internal organ involvement is not expected. Classification of the local scleroderma is made according to the size and localization of the skin changes. There are few different therapeutical options but there is no specific therapy for the localized scleroderma. Conclusion: Many data regarding disease features and treatment options in juvenile scleroderma are based on studies among adults. There is a striking need for multicentric, prospective studies among children with juvenile scleroderma, in order to elucidate some questions of clinical course and disease prognosis. Recent genetic studies have revealed the role of the genetic factors (namely HLA class II) in the pathogenesis of the disease. Emerging biological agents and new treatment options are showing promising results.

Viscosupplementation with intra-articular injections of hyaluronic acid is recommended as a second line treatment for knee OA, after failure of non-pharmacological modalities and usual pain killers. Nevertheless there are still controversies regarding clinical relevance of its effects. Research is looking for the best way to improve the performance of viscosupplementation in order to obtain a faster, longer-lasting and more pronounced effect. Antioxidants have been assessed in combination with hyaluronic acid because the injected hyaluronate is rapidly degraded by the reactive oxygen species, present in large amounts in the OA synovial fluid, limiting its residence time into the joint. Sorbitol and mannitol which have intrinsic free radical scavenger properties have been the most studied antioxidants. Sodium hyaluronate and polyols develop together a complex based on a dense network of hydrogen bonds which do not modify the visco-elsatic properties of hyaluronic acid. The oxygen free radicals neutralization by mannitol has been proven to delay the degradation of both linear and cross-linked HA in several in vitro models of oxidative stress. The antioxidant effect of these polyols may also play a role in accelerating onset of analgesia, as demonstrated in a double blind controlled trial comparing a mannitol-modified viscosupplement to regular hyaluronic acid. The addition of mannitol and sorbitol to hyaluronic acid does not alter the safety and local tolerability. In summary, adding a polyol to hyaluronic acid may improve the effects of viscosupplementation by reducing the rate of degradation of HA leading to a faster effect on pain relief without increasing the risk of adverse effect.

Background: The association of primary Sjogren´s Syndrome (SS) and amyloidosis has been recognized but scarcely assessed. Objective: Herein we analyzed the literature regarding this association in order to describe a SS phenotype prone to both conditions. Methods: PubMed and CINAHL databases were searched from inception until April 2016 for the keywords: Amyloidosis, amyloid, Sjögren's syndrome and their combinations. Results: Most of the cases corresponded to localized amyloidosis mainly at skin and lung, whereas only three cases were systemic. Other places of localized amyloidosis were the kidney, tracheobronchial, tongue, breast, salivary gland and vocal cord. Most of the localized forms corresponded to AL type, whereas AA amyloidosis was uncommon. The diagnosis of amyloidosis followed the SS onset (1-25 years) and in the majority of patients the presence of B cell hyperactivity traduced by hypergammaglobulinemia, positive rheumatoid factor and/or anti-Ro/SSA and anti-La/SSB antibodies were observed. In seven patients, an associated lymphoma was also documented. Conclusion: The spectrum of lymphoid proliferation associated with SS extends beyond the classical B cell lymphoma. Localized or systemic amyloidosis might coexist with primary SS. Localized amyloidosis should be suspected in patients with consistently high serologic activity and suggestive lesions.

Sjogren's syndrome (SS) is a T cell-mediated autoimmune disease of the systemic exocrine glands, such as salivary and lacrimal glands. A variety of T-cell subpopulations maintain immune tolerance in the thymus and periphery through complex immune responses including cellular and humoral immunity. The T-cell subpopulations exhibiting abnormal or unique phenotypes and impaired functionality have been reported to play important roles in the cellular mechanisms of autoimmunity in SS patients and animal models of SS. In this review, we focused on follicular helper T cells related to antibody production and regulatory T cells to control immune tolerance in the pathogenesis of SS. The unique roles of these T-cell subpopulations in the process of the onset or development of SS have been demonstrated in this review of recent publications. The clinical application of these T-cell subpopulations will be helpful for the development of new techniques for diagnosis or treatment of SS in the future.

Background: The strong association between HLA-B27 and spondyloarthritis (SpA) has demonstrated that typing the HLA-B27 antigen is a crucial step in diagnosis and aids in defining the progression and severity of disease. Objective: To describe the frequency of HLA-B27 in Colombian individuals with clinical manifestations associated with SpA. Materials and Methodology: We retrospectively analyzed 4109 HLA-B27 typing requests to the Hospital Militar Central and the Instituto de Referencia Andino from Colombian individuals with clinical signs suggestive of SpA between 2009 and 2012. We used basic digital cytometry followed by Polymerase Chain Reaction with sequence specific primers when confirmation was needed. We determined the frequency of HLA-B27 in the population and levels of association of HLA-B27 with SpA. Results: Our population included 1585 men (36.8%) and 2524 women (61.4%). The predominant age range was between 19 and 45 years (49.9%). The majority (95.4%) of the study population came from the Andean region and eastern plains. The most frequent clinical manifestations were peripheral. Only a small fraction (12.1%) of the 4109 subjects was HLA-B27 positive. Of those, 56.9% were male, and 54.7% were between 19 and 45 years old. In contrast, when rheumatologists referred the HLA B27, 64% were found to be positive. Conclusion: The frequency of the HLA-B27 allele in individuals with clinical signs suggestive of SpA was low, in accordance with the lower prevalence found in Colombian patients diagnosed with SpA compared to American and European population.

Introduction: Morphea is an uncommon disease that presents with skin induration and sclerosis. The disease is common in Caucasians and there are few studies describing the clinicoepidemiological profile of these patients from the Indian subcontinent. Methods: This prospective study was conducted during a three year period from June 2014 to May 2017. All patients of morphea presenting to the dermatology outpatient department were evaluated for parameters like age, sex, duration, age of onset, clinical subtype and possible disease associations and triggering factors. The data was analysed, tabulated and mean, standard deviation and percentages calculated. Results: 47 patients were incorporated into the study. These comprised of 10 (21.28%) males and 37 (78.72%) females. The average age of the patients was 23.92 ± 12.07 years with a mean age of onset being 22.13 ± 12.51 years. 22 (46.80%) patients presented within one year of onset of disease. Plaque morphea was the commonest type seen in 31 (65.96%) patients followed by linear morphea in 9 (19.15%) patients. We found preceding trauma in 3 patients and morphea developed following herpes zoster and intramuscular injection in one patient each. Conclusion: Morphea is an uncommon disease that is seen predominantly in females and young individuals. Circumscribed plaque morphea is the commonest variant. Certain predisposing factors like trauma can precede it.

Background: Rapid diagnosis of septic arthritis from Gram-stain microscopy is limited by an inherent false-negative rate of 25-78%. The presence of concomitant crystal arthritis in 5% of cases represents a particular diagnostic challenge. Objectives: This study aims to investigate the effects that a concomitant crystal arthropathy has on the ability of Gram-stain microscopy of synovial fluid to diagnose a septic arthritis. Methods: This is a 22-year retrospective cohort study. Inclusion criteria were a positive synovial fluid culture result with a positive clinical diagnosis of septic arthritis. Results were correlated with the presence or absence of urate and calcium pyrophosphate crystals, and Gram-stain result. During this time our collection and analysis methods remained unchanged. All samples were collected in Lithium Heparin containers. Chi-squared test with a p value < 0.05 was considered significant. Results: 602 synovial fluid samples were included. 162 cases of concomitant crystal arthritis were identified (27%). Of these, 16 (10%) had an initial negative Gram-stain. Out of the 440 samples with no crystals detected, 18 (4%) had an initial negative Gram-stain microscopy result (p < 0.05). Conclusion: The incidence of concurrent septic and crystal arthritis may be higher than previously thought. Synovial fluid samples in concomitant septic and crystal arthritis are significantly less likely to have a positive Gram-stain at microscopy than in cases of an isolated septic arthritis. We would advise the clinician to maintain a high index of suspicion for septic arthritis in these patients.

Objectives: to describe the clinical and paraclinical profile of our population with spondyloarthritis and to investigate factors associated with active and severe disease. Methods: A retrospective observational study, conducted in the rheumatology department at CHU Hassan II of Fez, analyzing the records of patients diagnosed as spondylarthritis during the period extending from January 2009 to June 2014. Patients having a spondyloarthritis associated with intestinal bowel disease and psoriasis, as well as reactive arthritis were excluded. Results: we included 136 patients (86 men and 50 women, the mean age was 38.06 ± 13.46 years, diagnostic delay was 6 years and 3 months). Patients with the HLA-B27 allele numbered 7. The clinical symptomatology was dominated by axial involvement in 92.6% of cases, followed by peripheral damage in 35.3% of cases and entheseal involvement in 30.1% of cases. Uveitis was the most common extra-articular manifestation in 18.4% of cases. 54% of patients had active disease, 53% had disease with functional impairment, and 34% had severe disease.10.3% of patients had juvenile-onset, 24.1% patients had a coxitis. We didn't find a significant association between sanitation (availability of drinking water and the use of refrigerator) the diagnosis delay, HLA-B27 allele, smoking, and activity and severity disease. A significant association was found between the presence of coxitis and male sex (p = 0.005). Conclusion: spondyloarthritis in our population still suffers from a delayed diagnosis with the frequency of active and severe forms.

Objective: To assess the intra- and inter-observer reliability of Ultrasound (US) in scoring B-mode, Doppler synovitis and combined B-mode and Doppler synovitis scores in different peripheral joints of Rheumatoid Arthritis (RA) patients. Methods: Four rheumatologists with a formal training in Musculoskeletal US (MSKUS) particularly focus on definitions and scoring synovitis on B-mode and Doppler mode participated in a patient- based reliability exercise on 16 active RA patients. The four rheumatologists independently and consecutively performed a B-mode and Power Doppler (PD) US assessment of 7 joints of each patient in two rounds in a blinded fashion. Each joint was semi quantitatively scored from 0 to 3 for B-mode Synovitis (BS), Doppler Synovitis (DS), and combined B-mode/Doppler synovitis (CS). Intraobserver reliability was assessed by Cohen's Κ. Interobserver reliability was assessed by unweight Light's Κ. Results: The mean prevalence of synovitis on B-mode was 83% of joints; scores ranging from grade 1 in 18% of joints, to grade 3 in 33%. In 55% of joints synovial PD signal was detected and the distribution of scores range from 14% of joints for grade 3, to 26% for grade 2. After a total of 448 joints scanned with 896 adquired images our intraobserver and interobserver reliability was good to excellent for most of the joints. Conclusion: Formal, structured and continuous training in musculoskeletal ultrasound would bring a good to excellent reproducibility in rheumatological hands with a high reliability in real time acquisition BS, DS and CS modalities for scoring synovitis in patients with active rheumatoid arthritis.

Introduction: Various psychiatric disorders, especially depression and anxiety, are seen in 2/3 of the chronic rheumatic diseases with chronic pain. In this study, we aimed to define anxiety and depression rates in Rheumatoid Arthritis (RA) and Ankylosing Spondylitis (AS) patients (under treatment) with similar age and gender; to compare the obtained data with each other and healthy control group; and also we aimed to investigate the relationship between human leukocyte antigen B27(HLA-B27) in AS, Rheumatoid Factor (RF) and anti-cyclic citrullinated peptide antibodies (anti-CCP) in RA with anxiety and depression. Method: 46 patients with RA, 43 patients with AS and 29 healthy volunteers were evaluated with Beck Depression Inventory (BAI) and Beck Anxiety Inventory (BAI). Participants were also noted for their educational status, occupation status, family history of illness, duration of the disease and their current treatments. Then we compared the obtained data with the healthy control group. SPSS (IBM Corp. Released 2012. IBM SPSS Statistics for Windows, Version 21.p=0. Armonk, NY: IBM Corp.) was used for performing statistical analysis. Results: There was no difference between the groups according to age, sex, duration of illness (p=0.104, p=0.767, p=0.377). A significant difference between groups in terms of BAI values were determined (p=0.018). In subgroup analyzes, the median BAI value of AS group was found to be higher than the control group (p=0.020). There were no differences in BAI values between AS and RA groups or between RA and the control groups (p>0.05, p>0.05 respectively). Also, there were no differences between the groups in terms of BDI values (p=0.055). Conclusions: Especially, chronic pain-related diseases are often associated with mental disorders, especially depression and anxiety. As a result, a multidisciplinary approach including psychiatric support should be used when planning treatment for these patients.

Background: Wallet neuritis is an example of extra-spinal tunnel neuropathy concerning sciatic nerve. Its clinical appearance often gets confused with sciatica of lumbar spine origin. Wallet- induced chronic sciatic nerve constriction produces gluteal and ipsilateral lower extremity pain, tingling, and burning sensation. It was Lutz, first describing credit-card wallet sciatica in an Attorney, surfaced on Journal of American Medical Association (JAMA), 1978; however, the condition has not been well-studied in various other occupations. Case Summary: In this write-up, we take the privilege of demonstrating wallet neuritis as an example of peripheral sensitization in three different professionals' namely specialist doctor, driver, and banker first time in Bangladesh. All the three patients' demonstrated aggravated gluteal pain with radiation on the homo-lateral lower extremity while remained seated on heavy wallet for a while, fortunately improved discontinuing such stuff with. Alongside radical wallectomy, piriformis stretching exercise on the affected side had also been recommended and found worthy in terms of pain relief. Conclusion: long-standing use of rear pocket wallet may compress and sensitize ipsilateral sciatic nerve, generating features resembling lumbago sciatica; thereby, remains a source of patients' misery and diagnostic illusion for pain physicians as well.

Background: A distal interphalangeal (DIP) joint involvement in the adult-onset Still's disease (AOSD) has been described in some publications but is rarely reported to be severe. We report severe DIP joints damages in a young patient with AOSD. Case Report: A 22 years old patient presented to our department complaining of inflammatory joints pain associated with prolonged fever and cutaneous rash. Physical examination identified polyarthritis and hepatosplenomegaly but no lymphadenopathies. After an extensive screening for neoplastic, infectious or hematologic diseases, the patient was finally diagnosed with AOSD. Treatment based on corticosteroids was then initiated with a good response on systemic signs. However, the patient continued to have recurrent arthritis affecting wrists and proximal interphalangeal joints. A Few years later, he developed a severe and disabling DIP arthritis with signs of joint destruction on conventional radiographs and MRI. Despite the initiation of methotrexate with optimal dosage, the patient continued to have polyarticular flares. The combination of methotrexate and sulfasalazine was responsible for drug-induced hepatotoxicity and this treatment was stopped. Anti-TNFα treatment was then indicated as general signs improved but severe joints damage persisted. Unfortunately, and due to healthcare system considerations, the patient was not able to benefit from TNFα inhibitors, and remained on methotrexate treatment only. Conculsion: The distal destructive arthritis during AOSD is rare and controversial. Our patient had a severe form with resistance to conventional therapies.