Search

Purpose of review Early access to rheumatology is imperative to achieve appropriate outcomes in rheumatologic diseases. But there seems to be a significant gap and disparity in the access to rheumatology care between urban and rural areas. This review was undertaken to analyze this issue. Recent findings A significant delay in diagnosis of rheumatic disorder has been correlated to the travel distance to rheumatologist. It is also clear that currently, a significant rheumatology workforce shortage exists and is projected to worsen significantly, thereby making this gap and disparity much bigger. Summary The scope of this gap and disparity in rheumatology care for rural patients remains incompletely defined and quantified. It is felt to be a significant issue and it is important to invest resources to obtain information about its scope. In addition, a number of solutions already exist which can be implemented using current network and infrastructure. These include relatively low-cost interventions such as patient navigator, remote rheumatology experts and if possible tele-rheumatology. These interventions can assist temporarily but a major improvement will require policy change at federal and state government level as well as involvement, buy-in, and incentivization of the providers and health networks providing rheumatology care.

Neuropsychiatric systemic lupus erythematosus (NPSLE) presents a diagnostic challenge as there is no unified pathophysiological process driving its presentation. Case reports are limited in detailing manifestations and outcomes of NPSLE. This case highlights a unique presentation of NPSLE and discusses challenges associated with diagnosis. A 27-year-old man with systemic lupus erythematosus presented with altered mentation. Initial laboratory results and computed tomography of the brain were unremarkable, but magnetic resonance imaging of the brain revealed ring-enhancing lesions reported as NCC. This led to an extensive infectious disease evaluation, but ultimately there was no evidence of infection. The patient was diagnosed with NPSLE; treatment with intravenous glucocorticoids and cyclophosphamide led to dramatic clinical improvement. Repeat brain magnetic resonance imaging showed resolution of the ringed lesions. This case illustrates the importance of thorough evaluation in immunocompromised patients and warns of the risk of anchoring bias that can lead to diagnostic delays.

Objective Due to an aging population, increasing prevalence of rheumatic disease, and a growing supply and demand gap of rheumatology providers, innovative solutions are needed to meet the needs of persons with rheumatic conditions. Nurse practitioners (NPs) and physician assistants (PAs) have been identified as a group of health professionals who could help address the workforce shortage. The Executive Committee of the Association of Rheumatology Health Professionals (ARHP), a division of the American College of Rheumatology (ACR), charged a task force to facilitate the preparation of NPs/PAs to work in a rheumatology practice setting. Methods The task force, consisting of private practice and academic rheumatologists, and NPs and PAs, from both adult and pediatric settings, conducted a needs assessment survey of current NPs and PAs to identify mechanisms for acquiring rheumatology knowledge. Through face-to-face and webinar meetings, and incorporating stakeholder feedback, the task force designed a rheumatology curriculum outline to enrich the training of new NPs and PAs joining rheumatology practice. Results Informed by the needs assessment data and stakeholders, an NP/PA rheumatology curriculum outline was developed and endorsed by the ACR Board of Directors for use by community-based and academic rheumatology practices, whether pediatric or adult, who desire to add NPs and PAs to their practice setting. Conclusion As rheumatology is facing workforce shortages, the ACR/ARHP rheumatology curriculum outline can be utilized to train NPs and PAs and create more efficient integration of NPs and PAs into rheumatology practice.

Objective To update evidence-based recommendations for the treatment of patients with ankylosing spondylitis (AS) and nonradiographic axial spondyloarthritis (SpA). Methods We conducted updated systematic literature reviews for 20 clinical questions on pharmacologic treatment addressed in the 2015 guidelines, and for 26 new questions on pharmacologic treatment, treat-to-target strategy, and use of imaging. New questions addressed the use of secukinumab, ixekizumab, tofacitinib, tumor necrosis factor inhibitor (TNFi) biosimilars, and biologic tapering/discontinuation, among others. We used the Grading of Recommendations, Assessment, Development and Evaluation methodology to assess the quality of evidence and formulate recommendations and required at least 70% agreement among the voting panel. Results Recommendations for AS and nonradiographic axial SpA are similar. TNFi are recommended over secukinumab or ixekizumab as the first biologic to be used. Secukinumab or ixekizumab is recommended over the use of a second TNFi in patients with primary nonresponse to the first TNFi. TNFi, secukinumab, and ixekizumab are favored over tofacitinib. Co-administration of low-dose methotrexate with TNFi is not recommended, nor is a strict treat-to-target strategy or discontinuation or tapering of biologics in patients with stable disease. Sulfasalazine is recommended only for persistent peripheral arthritis when TNFi are contraindicated. For patients with unclear disease activity, spine or pelvis magnetic resonance imaging could aid assessment. Routine monitoring of radiographic changes with serial spine radiographs is not recommended. Conclusion These recommendations provide updated guidance regarding use of new medications and imaging of the axial skeleton in the management of AS and nonradiographic axial SpA.

Immunotherapy with immune checkpoint inhibitors (ICIs) has become a well-established modality to treat a number of different malignancies, especially in cases with advanced stages and/or recurrent diseases. These agents have been associated with development of a variety of autoimmune disorders as immune-related adverse events (IRAEs or irAEs). This review focuses on development of vasculitis with use of ICI. Available information on vasculitis associated with immune checkpoint inhibition is limited primarily to case reports at this time. Most immune-related adverse events will not present as vasculitis, and it is an uncommon manifestation and/or is under-reported. There are no current well-established guidelines for treating vasculitis associated with ICIs; initial management would usually start with consideration of discontinuing the ICI and administering corticosteroids. Collaboration between treating oncologists and rheumatologists is necessary for a combined approach to management. While arthralgias, myalgias, and inflammatory arthritis frequently occur as irAEs, vasculitis is an uncommon presentation. Vasculitis has been reported with all of the available ICI agents, and there seems to be no clear difference in the risk based on small numbers. Large vessel vasculitis and vasculitis of the nervous system were the most commonly reported types of vasculitis but cases of vasculitis involving medium and small vessels have also been reported. It is challenging to know if the underlying disease or ICIs are the main culprit in development of vasculitis and requires a collaborative relationship between the treating oncologist and rheumatologist. Except in very mild cases, development of vasculitis during ICI therapy requires temporary or permanent discontinuation of ICI.

A 53-year-old male with rheumatoid arthritis presented with recurrent headaches, seizures and right-sided lower extremity paralysis while on antiepileptic medications. Work up revealed pachymeningeal and leptomeningeal enhancement on brain MRI. Differential diagnosis included a variety of infections, neoplasm and vasculitis. Histopathology showed findings consistent with rheumatoid meningitis (RM). Ultimately based on symptoms, MRI findings and tissue pathology, he was diagnosed with RM. Intravenous pulse dose steroids were initiated followed by rituximab every 6 months, resulting in significant improvement of the brain MRI findings. Patient has remained seizure free.

Understanding of the pathogenesis of inflammatory bowel disease (IBD) continues to evolve. Current evidence suggests that IBD results from an inappropriate inflammatory response to intestinal microbes in a genetically susceptible host. Dysbiosis, that is, unfavorable alteration of the composition and function of gastrointestinal (GI) microbiota or chronic pathogen infection, may be the mechanisms microbiota contribute to the pathogenesis of IBD. Several microbes have been associated with IBD, and among them, bacteria such as Clostridium species, gram-negatives, fecal microbes, and Mycobacterium avium subspecies remain exciting candidate pathogens but yet undiscovered other bacteria, viruses, and fungi also likely contribute significantly. Current therapeutic strategies targeting microbes that may be beneficial include probiotics and possibly fecal microbiota transplantation while dietary changes, prebiotics, and antibiotics have proven to be unhelpful. Newly discovered techniques focusing on molecular analysis of gut bacteria flora in combination with high genomic approaches are likely to further the insights into the role that microbes play in the development of IBD.

Objective Fertility is reduced in patients with Rheumatoid Arthritis due to unknown cause. Few studies have addressed pregnancy outcomes in RA. This study was undertaken to determine the frequency of complications occurring during pregnancy for women with RA and compare with the general obstetric population by using the largest inpatient care database. Methods By using the 2003–2011 Nationwide Inpatient Sample of Healthcare Cost and Utilization Project, we estimated the number of obstetric hospitalization in women with RA between the age group 18–50 years. Demographic characteristics and in-hospital obstetric complications for all pregnancy-related admissions for women with and without RA were compared. Multivariate logistic regression analysis was used to obtain adjusted odds ratio. Results The total number of obstetric hospitalization was 42.32 million of which 31,439 were women with RA. The maternal age of RA population was higher (30.5 years) than that in the control group (27 years). After adjusting for potential confounders, maternal RA population had a significantly higher prevalence of hypertensive diseases, premature rupture of membranes, antepartum hemorrhage, preterm delivery, intrauterine growth retardation and cesarean delivery. The prevalence of postpartum hemorrhage and the risk of inpatient mortality were not different between two groups. Conclusion Women with RA have a higher risk of adverse outcomes of pregnancy and thus close antenatal and post-delivery monitoring need to be performed in order to reduce complications. Further studies are needed to examine these findings in relation to severity of disease, medications used and the presence of other comorbidities.

Osteoporosis is a condition generally associated with olderwomen, but it is rapidly becoming a growing problem for males as well. Screening and treating men early is the only way to address this problem. The known demographic factors of osteoporosis in males such as age, race and BMI as well as secondary causes oflow bone mineral density (BMD) i.e. osteoporosis, have not been well examined in the actual practice settingbased on available literature. This study aims to describe the prevalence of the demographic factors and secondary causes in men with low BMD and also to assess their individual contribution to the overall prevalence. A retrospective chart review of 585 men who underwent bone density scan at the University of Mississippi Medical Center from 2005-2012 was performed. At the time of their scans, patients were also asked to complete a questionnaire assessing demographics, comorbidities, social factors, and medication use. The results suggest that racial difference and differences in secondary causes exist in the epidemiology of male osteoporosis, and this needs to be assessed further. The notion that African American males are protected from OP is unsupported in our data as well as the literature. Overall our research demonstrated that low BMI is the most important factor associated with low BMD in male patients.

Background/purpose Venous thromboembolism (VTE) is a major cause of morbidity and mortality in hospitalised patients particularly patients with autoimmune disorders. Nationwide Inpatient Sample (NIS) database was analysed to determine trends in rate of hospitalisation and mortality from VTE in hospitalised systemic lupus erythematosus (SLE) patients to assess its impact. Methods 2003–2011 NIS database of Healthcare Cost and Utilisation Project was queried to identify all adults (age >18 years) hospitalised with SLE and VTE. Demographic characteristics and in-hospital outcomes of this population were compared to SLE patients without a VTE diagnosis. Multivariate logistic regression analysis was used to obtain adjusted odds ratio (OR). Results The total number of hospitalised patients with SLE was 2 99 595 of which 9175 (3.06%) had VTE. Mean age of the study population was 50 years and 89% were females. Mean age of SLE patients with VTE was lower than those without VTE (48 vs 51 years). Rate of VTE was higher in African Americans as compared to Caucasians (3.8% vs 2.8%) and in males when compared to females (4.3% vs 2.9%). After adjusting for potential confounders, compared to those without VTE, SLE patients with VTE had higher inpatient mortality (5% vs 2.0%, OR 2.35 (CI: 2.10 to 2.62, p The rate of VTE among SLE patients have increased since 2005 as shown in Graph 1. Conclusion VTE in hospitalised patients with SLE is associated with significantly higher inpatient mortality, greater disability at discharge, increased length of stay and higher cost of hospitalisation. The rate of VTE in SLE patients is on rise likely due to awareness and higher reporting. In this database patients with SLE and VTE were younger. Male sex and African-American race may be associated with an increased risk of VTE in patients with SLE. This cross-sectional study would help in the development and implementation of appropriate prophylactic strategies in high risk SLE population.

Background Systemic Lupus Erythematosus (SLE) may increase this risk of acute pulmonary embolism (PE) and its complications in hospitalised patients. Herein, Nationwide Inpatient Sample (NIS) database from 2003–2011 was analysed to assess the relationship of PE and SLE in hospitalised patients. Methods NIS database (2003–2011) was queried to identify all adults (age >18 years) with SLE and PE using appropriate ICD-9 codes. Demographic characteristics and in-hospital outcomes were compared between SLE patients with and without a PE. Multivariate logistic regression analysis was used to obtain adjusted odds ratio (OR). Results Of 2 99 595 hospitalizations of patients with SLE from 2003–2011, 3839 (1.28%) had PE, 1.83 times of the background prevalence with OR 1.85 (p In comparison to 10-year-analysis of National Hospital Discharge Survey (NHDS) database, the results show similar overall increase in risk of developing PE with SLE, mean age, sex ratio, length of stay, higher risk in African-Americans and increased mortality except, in NIS database, PE was more common in males not females (table 2). Conclusion SLE significantly increases the risk of developing PE in hospitalised patients. Furthermore, PE with SLE is associated with significantly higher mortality and cost of hospitalisation, increased LOS and greater disability at discharge. These results also suggest that African-Americans may have a higher risk of PE but role of sex needs further evaluation. These results suggest thromboembolism prophylaxis should be considered in hospitalised SLE patients but more studies are needed to further elucidate the relationship and risk of PE in SLE, especially in hospitalised patients.

Objective To provide evidence-based recommendations for the treatment of patients with ankylosing spondylitis (AS) and nonradiographic axial spondyloarthritis (SpA). Methods A core group led the development of the recommendations, starting with the treatment questions. A literature review group conducted systematic literature reviews of studies that addressed 57 specific treatment questions, based on searches conducted in OVID Medline (1946–2014), PubMed (1966–2014), and the Cochrane Library. We assessed the quality of evidence using the Grading of Recommendations, Assessment, Development and Evaluation (GRADE) method. A separate voting group reviewed the evidence and voted on recommendations for each question using the GRADE framework. Results In patients with active AS, the strong recommendations included use of nonsteroidal antiinflammatory drugs (NSAIDs), use of tumor necrosis factor inhibitors (TNFi) when activity persists despite NSAID treatment, not to use systemic glucocorticoids, use of physical therapy, and use of hip arthroplasty for patients with advanced hip arthritis. Among the conditional recommendations was that no particular TNFi was preferred except in patients with concomitant inflammatory bowel disease or recurrent iritis, in whom TNFi monoclonal antibodies should be used. In patients with active nonradiographic axial SpA despite treatment with NSAIDs, we conditionally recommend treatment with TNFi. Other recommendations for patients with nonradiographic axial SpA were based on indirect evidence and were the same as for patients with AS. Conclusion These recommendations provide guidance for the management of common clinical questions in AS and nonradiographic axial SpA. Additional research on optimal medication management over time, disease monitoring, and preventive care is needed to help establish best practices in these areas.

Data on coexisting Graves' disease (GD), hypoparathyroidism, and systemic lupus erythematosus (SLE) are limited. The thyroid and parathyroid glands may be extra sensitive to irradiation damage in an underlying autoimmune condition. A 34-year-old black woman presented with tetanic-like cramps, easy skin bruising, fatigue, weight gain, nocturia and back pain. She was previously diagnosed with GD in 2001 and underwent radioiodine therapy (RAI) in 9/01 using 6 mCi. PostRAI (November 2001) she developed hypocalcemia and hypothyroidism (2/02). In 2007, SLE was diagnosed. In October 2009, s-calcium and PTH were still low at 7.1 mg/dl and 9 pg/mL, respectively, although the patient denied symptoms on vitamin D and calcium supplementation. To identify possible autoimmune damage of the parathyroids, we evaluated the presence of activating antibodies to the CaSR and also analyzed the DNA sequence of all 6 translated exons and flanking intronic sequences of her CaSR gene for a functionally significant CaSR mutation but neither was positive. The initial autoimmune damage to her thyroid and possibly parathyroid glands followed by irradiation of them seems to have contributed to her developing both hypoparathyroidism (11/01) and hypothyroidism (2002). The patient could potentially have had parathyroid autoantibodies in 2001 that disappeared by 2009 when she was tested for them. We consider that the multiple autoimmune conditions developed over the past decade of her life with the concurrent irradiation contributing to her brittle hypoparathyroidism. Select patients with GD and perhaps parathyroid autoantibodies with a slowly developing destructive impact on the parathyroid glands may then develop overt hyoparathyroidism with rather low dose RAI ablation. This patient adds to the evolving spectrum of polyglandular syndrome variants.

Neuromyelitis optica (NMO) and neuromyelitis optica spectrum disorders (NMOSD), previously known as Devic’s syndrome, are a group of inflammatory disorders of the central nervous system (CNS) characterized by severe, immune-mediated demyelination and axonal damage, predominantly targeting optic nerves and the spinal cord typically associated with a disease-specific serum NMO-IgG antibody that selectively binds aquaporin-4 (AQP4). The classic and best-defined features of NMOSD include acute attacks of bilateral or rapidly sequential optic neuritis (leading to visual loss) or transverse myelitis (often causing limb weakness and bladder dysfunction) or both with a typically relapsing course. The diagnosis of NMO/NMOSD requires a consistent history and examination with typical clinical presentations, findings on spinal cord neuroimaging with MRI, cerebrospinal fluid analysis along with determination of AQP4-IgG serum autoantibody status, and exclusion of other disorders. Two major advances in this field has been the development of diagnostic criteria and treatment recommendations. Consensus diagnostic criteria have been established and were recently revised and published in 2015, enhancing the ability to make a diagnosis and appropriately evaluate these disorders. Expert recommendations and uncontrolled trials form the basis of treatment guidelines. All patients with suspected NMOSD should be treated for acute attacks as soon as possible with high-dose intravenous methylprednisolone −1 gram daily for three to five consecutive days and in some cases, plasma exchange should be used. It is recommended that every patient with NMOSD be started on an immunosuppressive agent, such as, azathioprine, methotrexate, or mycophenolate and in some cases, rituximab, soon after the acute attack and usually be treated for about 5 years after the attack. These advances have helped improve the prognosis and outcome in these disorders.

Anti-neutrophil cytoplasmic antibodies (ANCA) have two common patterns detected by indirect immunoXuorescence test (IIF test). The cytoplasmic pattern (c-ANCA) that is strongly associated with antibodies against proteinase-3 (PR3) and the perinuclear pattern (p-ANCA) that is mostly directed against myeloperoxidase (MPO). Anti-proteinase 3 and anti-MPO are characteristic for pauci-immune small vessel vasculitis like Wegener’s granulomatosis, microscopic polyangiitis, Churg–Strauss syndrome and idiopathic crescentic glomerulonephritis [1]. In Wegener’s granulomatosis (WG), the combination of c-ANCA with anti-PR3 antibodies has a sensitivity of 58% and a speciWcity of 99% [2]. On the other hand, the presence of c-ANCA (anti-PR3) in immune-complex-mediated vasculitis such as Henoch–Schonlein purpura (HSP) is very unusual. We report a case of biopsy proven HSP associated with IgG c-ANCA (anti-PR3).

Accurate diagnosis of rheumatoid arthritis may be difficult early in its course and demands high clinical suspicion, astute examination, and appropriate investigations. Early use of disease-modifying antirheumatic drugs and biologics has improved outcomes but requires close monitoring of disease course and adverse events.

Objectives. Mycophenolate mofetil (MMF) is a new immunosuppressive agent currently being used for the prevention of renal allograft rejection. MMF is a specific inhibitor of lymphocytes and is well tolerated leading to its use in other autoimmune diseases. We have used MMF for the treatment of seven patients with inflammatory myopathy and are hereby reporting our results. Case series. All of our patients were females (age 17–65 yr). They were symptomatic upon presentation and met classification criteria for idiopathic inflammatory myopathy. Inflammatory markers such as erythrocyte sedimentation rate and C-reactive protein as well as creatine kinase were significantly elevated in all the patients, indicating active disease. Corticosteroids were concomitantly being administered (20–60 mg/day of prednisone). Initial therapy with conventional immunosuppressives was either ineffective or had significant adverse effects leading to their discontinuation. MMF was started in doses of 500 mg twice a day and titrated up to 1 g twice a day. Results. Our patients have exhibited an impressive serological response to therapy with MMF and six patients had a marked improvement in their weakness. One patient had incomplete improvement in her weakness and has required additional therapies. MMF has been well tolerated during the treatment period (12–36 months). Conclusion. A striking clinical and laboratory response of active myositis in six out of seven patients in this series illustrates that MMF can be effectively used in management of autoimmune inflammatory myopathy and may be a suitable alternative to the conventional immunosuppressive agents.

In contrast to what is observed in the general population, a low body mass index (BMI) has been associated with accelerated mortality in patients with rheumatoid arthritis (RA). The aim of this study was to assess whether weight loss might explain these seemingly paradoxical observations.Our study included patients identified from the Veterans Affairs (VA) RA Registry. Dates of death were abstracted from VA electronic medical records. The BMI at each study visit and the change from the previous visit were determined. The maximum BMI of each patient was also obtained from medical records. The annualized rate of BMI loss was determined from the slope of change (per year) in BMI over visits within the preceding 13 months. Cox multivariable proportional hazards models were used to assess associations between BMI measures and mortality.In a sample of 1,674 patients, 312 deaths occurred over 9,183 person-years. A loss in BMI of ≥1 kg/m(2) was associated with a greater risk of death, after adjustment for demographics, comorbidities, BMI, smoking, and RA therapies (hazard ratio [HR] 1.99, 95% confidence interval [95% CI] 1.53-2.59, P 0.001). This association remained significant in a subsample analysis adjusting for C-reactive protein and physical function (HR 1.81, 95% CI 1.36-2.41, P 0.001). Weight loss at an annualized rate of ≥3 kg/m(2) was associated with the greatest risk of death (HR 2.49, 95% CI 1.73-3.57, P 0.001). Low BMI (20 kg/m(2) ) in patients with a history of obesity (30 kg/m(2) ) was associated with the greatest risk (HR 8.52, 95% CI 4.10-17.71, P 0.001).Weight loss is a strong predictor of death in patients with RA. These observations may explain the observed obesity paradox and do not support a biologically protective role of obesity.

Patients naïve to the Bath Ankylosing Spondylitis Disease Activity Index (BASDAI) and to the Ankylosing Spondylitis Disease Activity Score (ASDAS) have voiced confusion in our clinics over the use of the term "AS" in these instruments. It is unknown whether these tools may be applied to other related forms of spondyloarthritis (SpA). The Bath Ankylosing Spondylitis Functional Index (BASFI) questionnaire also requires more definitive validation. We 1) validated the BASFI against a standard definition of disability; and 2) validated slightly modified versions of the BASDAI and ASDAS questionnaires that replace references to "AS" with the term "inflammatory arthritis" for use in non-AS SpA.Adult patients with SpA enrolled in the Veterans Affairs Program to Understand the Longterm outcomes in Spondylo-ARthritis (PULSAR) completed the BASFI, BASDAI, ASDAS and altered versions of the BASDAI (PULSAR-modified Bath Disease Activity Index [PuBaDAI]) and ASDAS (PULSAR-modified Ankylosing Spondylitis Disease Activity Score [PuASDAS]). Spearman correlations and logistic regression were used to analyse the scores.The correlation between BASDAI and PuBaDAI and between ASDAS and PuASDAS scores was high (Spearman's rho=0.92, p0.001 and Spearman's rho=0.85, p0.001, respectively). The test-retest correlation of BASFI was also high (Spearman's rho=0.92, p0.001). The BASFI (OR 1.67, 95% C.I. 1.12-2.47), ASDAS (OR 1.34, 95% C.I. 1.02-1.76) and PuASDAS (OR 1.62, 95% C.I. 1.07-2.49) predicted federally-determined disability.Preliminary data suggest that BASDAI and ASDAS scores correlate well with modified forms of these questionnaires and that the ASDAS, PuASDAS and BASFI are associated with disability.

Background The actual incidence and prevalence of the various histological classes (based on World Health Organization classification) of lupus nephritis (LN) are not known but seem to vary with sex, age, and ethnicity. We have analyzed renal biopsies in patients with systemic lupus erythematosus (SLE) at our center, and hereby report our experience. Methods All renal biopsies performed at the University of Mississippi between January 1999 and December 2004 in patients with SLE were retrospectively analyzed. Results were validated by a detailed review of renal biopsy reports and additional records were reviewed for data specific to LN disease activity. Results There were 92 renal biopsies performed in patients with SLE during a 6-year period. These included 84 African Americans (72 women and 12 men), 5 whites (4 women and 1 man), and 3 unknown race (1 F, 2 M) subjects. The prevalence of LN classes in our cohort was as follows: class I (0%), class II (9.8%), class III (8.7%), class IV (36.9%), class V (40.2%), and class VI (4.3%). Prevalence of class V LN among males was high at 40%. Conclusions In contrast to previous literature, isolated membranous lupus nephritis (MLN) was much more prevalent in this series—40% versus 14%. Also, no sex difference in the prevalence of MLN was seen. This biopsy cohort suggests that MLN/ class V disease may be more common than previously reported especially in African American population.

Background Devic’s syndrome or neuromyelitis optica (NMO) is an inflammatory demyelinating disease of the central nervous system associated with optic neuritis, myelitis involving 3 or more contiguous spinal cord segments, and seropositivity for NMO-IgG antibody. Case A 22-year-old African American woman P1G0 at 22 weeks of gestation presented with weakness for 1 week. The weakness initially started in the left lower extremity and then involved the other extremities. She also had horizontal diplopia, temporal headache, and arthralgias. On physical examination, she had a discoid rash behind the left ear, muscle strength 3/5 in the upper and 0/5 in the lower extremities, and hyporeflexia. She had lymphopenia, a highly positive antinuclear antibody, and SS-A/ SS-B antibodies. The magnetic resonance imaging of the patient showed abnormal cord signal within brain and cervical and thoracic spine. Salivary gland biopsy revealed mild lymphoplasmacytic inflammation. The NMO antibody was positive. A diagnosis of Devic’s syndrome associated with probable systemic lupus erythematosus (SLE) was made. She was treated with pulse IV solumedrol and plasmapheresis for 4 days. The patient improved clinically with treatment, but the fetus developed bradycardia, which was treated with IV dexamethasone. Discussion and Conclusion There is a debate about the relationship of NMO with autoimmune disorders, such as SLE or Sjogren syndrome. If clinically evident SLE or Sjogren or positive autoantibodies coexist with NMO signs and symptoms, the neurologic process could be an independent association due to NMO or may be a vasculitic complication of the systemic disease. Our case highlights these issues, difficulty in making a correct diagnosis, and choosing the appropriate management. Further case studies are needed to explore these important issues.

Reiter's syndrome is one of the reactive forms of seronegative spondyloarthropathies. Various therapies used in the management of Reiter's syndrome are nonsteroidal antiinflammatory drugs (NSAIDs), antibiotics, and disease-modifying antirheumatic drugs (DMARDs) such as sulfasalazine (SSZ) or methotrexate (MTX). There is only one case report of successful treatment of Reiter's syndrome with tumor necrosis factor-alpha (TNF-alpha) blockers in human immunodeficiency virus (HIV) patient (Gaylis N, 2003, J Rheumatol 30(2):407-411 Feb). We hereby report a case of Reiter's syndrome treated successfully with infliximab, an anti-TNF-alpha chimeric monoclonal antibody. A 28-year-old white male presented with painful swelling of right elbow and ankle joints, urethritis. and lesions involving skin of soles of feet and penis. Detailed work-up of sexually transmitted diseases (STDs), HIV, and systemic etiology were negative. Despite aggressive treatment with antibiotics, NSAIDS, prednisone, and MTX for 3 months, he had persistent synovitis and worsening of skin lesions. He was then treated with infliximab 200 mg intravenously at weeks 0, 2, 6, and 14 weeks which resulted in complete resolution of arthritis and skin lesions within 6 weeks of infliximab therapy.

Nephrogenic fibrosing dermopathy is a unique fibrosing disorder recently identified to occur exclusively among patients with renal disease. The cutaneous findings are similar to those of systemic sclerosis, but it is important to differentiate between these two disorders because of significant prognostic and therapeutic implications. Nephrogenic fibrosing dermopathy is usually a diagnosis of exclusion, but the condition does have distinct clinical and histopathologic findings. It appears to be multifactorial in pathogenesis, and no specific cause has been identified. No specific treatment modality has been consistently effective, but there have been reports of improvement that occurred either spontaneously with renal recovery or after renal transplantation. We present an interesting case of a 33-year-old woman diagnosed with nephrogenic fibrosing dermopathy, along with a review of the literature.

Libman-Sacks Endocarditis (LSE) affects patients with systemic lupus erythematosus (SLE) and positive antiphospholipid antibodies. These sterile verrucous vegetations typically affect left-sided heart valves and can cause valvular dysfunction and embolization. We present a case of a 42 year old

Systemic lupus erythematosus (SLE) is a chronic, relapsing, and remitting disease affecting primarily African American females of child bearing age. Familial aggregation of this disease suggests that at least part of the susceptibility for this disease is genetic, although environmental and hormonal influences are also likely to play a role. Early studies of genetic susceptibility to SLE revealed several of the major histocompatibility complex molecules, namely HLA DR, to be linked to SLE. Meta-analysis of genome scans has yielded loci significant for lupus patients, one of which includes the MHC region. Regulatory T cells are immunoregulatory cells that modulate activated immune cells. These cells play a large role in homeostasis of the immune responses and maintenance of immunologic tolerance, i.e., prevention of autoimmunity. Decreased numbers of regulatory T cells have been described in many autoimmune diseases, including systemic lupus erythematosus. Autoantibody production in systemic lupus erythematosus and the resulting immune complex formation and complex deposition into tissues are arguably the central core of immune dysregulation leading to disease manifestations and symptoms. Inability of the immune system to recognize and inhibit autoreactive immune cells in this particular autoimmune disease may be the result of inappropriate numbers and function of regulatory T cells. This study aims to characterize the immune cell population in patients from our community suffering from systemic lupus erythematosus and to prove that these patients exhibit a unique cellular profile compared to healthy age, race and gender matched control subjects. Surprisingly, our findings demonstrate that patients from the local Mississippi area exhibit increased proportions of CD25(+) FoxP3(+) regulatory T cells and CD25(+) FoxP3(-) T cells (of CD45(+) CD3(+) CD4(+) helper T cells) as compared to healthy controls. HLA tissue-typing of these lupus patients revealed a prominent subgroup (~30%) of patients possessing the HLA DRB1*1503 allele. The investigation of this subgroup demonstrated regulatory T cell composition similar to that of the total lupus group and to that of the non-HLA DRB1*1503 subgroup. Genetic analysis for molecular gene expression levels of various lupus-associated genes by real-time PCR demonstrated a unique profile as compared to healthy controls. Increased gene expression of FoxP3 together with decreased gene expression levels of GATA3, TNFAIP3, and TNFSF4 suggest that variations in gene products compared to healthy controls may be playing a role in the immune cell dysregulation and disproportionate CD25(+) FoxP3(+) regulatory T cells.

Testicular involvement in connective tissue diseases (CTD) is typically caused by medium vessel vasculitis as in polyarteritis nodosa. Systemic lupus erythrematosus (SLE) and systemic sclerosis cause small vessel vasculitis, which is an unusual cause of orchitis. We hereby report a case of orchitis in a 28-year-old patient caused by vasculitis related to his lupus/scleroderma overlap CTD. He had an excellent response to steroids and azathioprine with complete resolution of his testicular and systemic symptoms. Our case highlights that although testicular involvement secondary to small vessel vasculitis in CTD is uncommon, it is still possible and should be evaluated.

Rheumatoid arthritis (RA) in the geriatric population presents a unique challenge to treating clinicians. It can present as preexisting disease that may have been present for years or as a de novo onset of the illness. Diagnosis and management requires a detailed knowledge of the disease, its differential diagnoses, and the therapeutic options. A number of other diseases can mimic the illness and must be thoroughly evaluated to avoid serious consequences. New agents to treat RA are available that have shown promise in clinical trials and practice. Aggressive RA treatment should not be withheld in the geriatric population just because of advanced age, rather, treatment should be individualized, especially considering comorbidities and other factors that can specifically affect a patient's quality of life. Coordination of care among geriatricians and rheumatologists is the key to achieving optimal outcome.

Sarcoidosis is a multisystemic inflammatory disorder of unknown etiology. It can present in various clinical forms. Involvement of muscles is common but isolated muscle mass, the only initial presenting complaint has never been reported. We report a 55-year-old white female who presented with a muscle mass, with no other clinical features of sarcoidosis. She was later found to have hilar lymphadenopathy and muscle biopsy confirmed the diagnosis of sarcoidosis. During clinical follow up patient remained stable without any medical intervention.

Cocaine use can be associated with a wide spectrum of rheumatic manifestations. It poses a diagnostic challenge as the patients usually withhold the information of cocaine use, and no serological tests are available to establish this diagnosis. We report a patient with vasculopathic syndrome secondary to cocaine use. Despite initial denial of drug abuse, skin biopsy suggested the diagnosis, which was subsequently confirmed by urine drug testing. Differentiating cocaine-associated pseudovasculitis from true vasculitis is necessary, as conventional treatment is usually ineffective without complete abstinence from cocaine use and may be associated with significant morbidity as well as mortality.

Although neurological involvement occurs in about 10% of patients with mixed connective tissue disease (MCTD), acute transverse myelitis (TM) has only been described in seven cases of MCTD. We hereby report a case of 70-year-old white female with transverse myelitis complicating her underlying MCTD. Our patient presented with lower extremity weakness, loss of sensation and incontinence one year after her diagnosis of MCTD. Her work-up revealed an abnormal MRI, with findings consistent with TM. She had an excellent response to initial therapy with six cycles of monthly intravenous immunoglobulins and steroids, with subsequent maintenance on azathioprine. She had a good neurological recovery with mild residual sequelae only. On basis of this case report and review of literature, we recommend ongoing surveillance and reporting of this rare neurological presentation in MCTD.

Introduction Fibromyalgia syndrome (FMS) is a disorder characterized by widespread muscloskeletal pain, stiffness, nonrestorative sleep, and fatigue. These patients have significant and unremitting fatigue, but currently none of the available medicines work well for its treatment. Modafinil has been used in other chronic syndromes associated with fatigue, such as multiple sclerosis and a few cases of FMS. Its use is indicated in narcolepsy and shift work sleep disorder (SWSD), and it is used as an adjunctive therapy for obstructive sleep apnea/hypopnea syndrome (OSAHS). Case Series We used modafinil in three patients with FMS who had severe fatigue and report our experience here. All of the patients in this series were females (ages 53, 58, and 63 years), met 1990 American College of Rheumatology (ACR) criteria for FMS, and had severe/disabling fatigue. Modafinil was initially started at 100 mg/d in the morning and subsequently increased to 200 mg/d. Two of the three patients had an excellent improvement of fatigue and one had moderate improvement as measured by the Modified Health Assessment Questionnaire (MDHAQ) and visual analogue scale (VAS) performed serially at 0-, 3-, and 6-month intervals. In the first patient, fatigue decreased from 10/10 to 3 and 2 on VAS at 3 and 6 months and was followed for 24 months. In the second patient, fatigue decreased from 10/10 to 1 and 2 at 3 and 6 months on the VAS and was followed for 18 months. In the third patient, fatigue decreased from 9/10 to 5 and 6 at 3 and 6 months on the VAS and was followed for 12 months. This improvement also translated into better overall well-being and functional status of these patients by their history and as measured by MDHAQ. All three patients were on modafinil 200 mg/d, with an average duration of follow-up of 18 months. Conclusion This case series suggests that modafinil may be a pharmacologic option in the treatment of fatigue associated with FMS and may prove useful in treating this disabling and difficult to treat symptom. Further studies should be done to confirm the preliminary findings of this case series and other case reports.

Introduction Cocaine use can present with a number of rheumatic manifestations. The diagnosis is often missed or delayed, as the history of abuse is denied, there are no specific signs of abuse, and available serological tests cannot establish this diagnosis. We hereby report a patient with cocaine use presenting with a vasculopathic syndrome. Case Report A 35-year-old AA female with history of CHF due to mitral stenosis and previous DVT presented with a 2-day history of skin rash on both legs. History and review of systems was otherwise unremarkable. She specifically denied use of new medications, recent infections, alcohol or drug abuse. Examination revealed diffuse palpable purpura in a reticular pattern on lower extremities, buttocks, elbows, and forearms, with blisters and desquamation. Systemic examination had no other pertinent findings. Laboratories revealed normal CBC, chemistry, urinalysis, complements, elevated LFTs, ESR of 3, and CRP of 4.7. Detailed work-up including hepatitis panel, HIV, ANA, ANCAs, APL antibodies, lupus anticoagulant, cryoglobulins, multiple blood and urine cultures, and hypercoagulability work-up was negative. Echocardiogram showed no evidence of vegetations. Urine drug screen was positive for cocaine and she admitted to its recent use when confronted. She was treated with prednisone 60 mg/day with improvement in rash and LFTs. The skin biopsy showed fibrin thrombi occluding vessels, extensive hemorrhage, and mild leukocytoclasis. The final report was vasculopathic reaction pattern with cocaine use in differential diagnosis. She was discharged on tapering doses of prednisone and counseled to abstain from cocaine. The final diagnosis was cocaine-associated pseudovasculitis. Discussion and Conclusion Cases with varied rheumatic manifestations associated with cocaine use have been described including ANCA +ve vasculitis-like syndromes mimicking Wegener9s, p-ANCA +ve necrotizing granulomatous vasculitis, HSP with necrotizing vasculitis, Churg-Strauss and urticarial vasculitis. Cases with +ve p- and c-ANCAs but with disparity to anti-MPO or PR3 specificities have been reported. In our patient, all the work-up including ANCAs was negative. A correct diagnosis is essential as some cases treated with steroids and immunosuppressives had a fatal outcome or persistent symptoms, but abstinence from cocaine resolved the symptoms. This case highlights the fact that there are no specific tests to correctly diagnose cocaine-induced vasculitis and only a high clinical suspicion and urine/serum drug screens can be helpful in such presentations.

Purpose Systemic lupus erythematosus (SLE) is an autoimmune disease of unknown etiology that affects young black women more severely than white women. Cytokines are a crucial factor in initiating, maintaining, and subsequently down-modulating the immune response in autoimmune disease. Significant ethnic differences in cytokines have not been reported. We investigated ethnic differences in TH1, TH2, and inflammatory cytokines in black and white women with recent-onset SLE. Methods Healthy premenopausal women with SLE for less than 5 years were enrolled. A standardized disease activity measure (Systemic Lupus Activity Measure or SLAM) was performed, and a blood sample was taken to measure cytokine levels by ELISA. We measured the inflammatory cytokines IL-1b, IL-6, and TNFa; the TH1 cytokines IL-2 and IFNg; and the TH2 and anti-inflammatory cytokine IL-10. BD Biosciences assays were used, had less than a 15% inter- and intra-assay variability in our hands, and recovered 90-105% of spiked cytokine. Results Thirty-three black and 14 white women were enrolled. The black women were slightly younger (31.6 vs 38.8 yr, p > .05) but clinical disease was similar. ACR criteria, SLAM score, and patient and MD global assessment were similar. TNF was not different between black and white women. Higher levels of IFNg (25.7 6 22.4 vs 18.3 6 18), IL-1 (31.2 6 44.1 vs 18.6 6 38.9), IL-2 (45.1 6 92.8 vs 12.1 6 9.5), and IL-6 (27.8 6 61.6 vs 10.2 6 10.6) were present in black than white women, respectively, but was not significant (p > .05). IL-10 was significantly lower in black (10.7 6 18.6) compared to white (29.8 6 25.4, p = .001) women. Conclusion The propensity of young black females to earlier onset and more severe SLE may be due in part to the inadequate anti-inflammatory TH2 cytokine IL-10 in the presence of an enhanced inflammatory and TH1 cytokine profile.

Introduction Reiter9s syndrome is one of the reactive forms of spondyloarthropathies. The clinical syndrome consists of a triad of asymmetric oligoathritis, urethritis, and conjunctivitis. It is also associated with characteristic mucocutaneous lesions. Various therapies used in the management are NSAIDs, antibiotics, and DMARDs such as SSZ or methotrexate. There is only 1 case report of successful treatment of Reiter9s syndrome with TNF-a blockers in an HIV+ patient. We report a case of Reiter9s syndrome treated successfully with infliximab. Case Report A 28-year-old white male presented with painful swelling of right elbow and ankle joints, urethritis, and lesions involving skin of soles of feet and penis. No other joint was involved. He also complained of fatigue, fever, and mild sore throat, but, otherwise, his review of systems, past, social and family history was negative. He was sexually active with one partner and denied any history of sexually transmitted disease. Pertinent positives on physical examination were synovitis of right elbow and ankle joints. He had extensive skin lesion on soles and penis consistent with keratoderma blennorrhagica and circinate balanitis. Laboratory workup was positive for a high WBC count of 13,700 cells/mm3, ESR of 82, and CRP of 11.1. Detailed workup of STDs, HIV, and systemic etiology was negative. Despite aggressive treatment with antibiotics, NSAIDS, prednisone, and methotrexate, he had persistent synovitis and worsening of skin lesions. Due to severity and resistance of disease to the conventional treatment modalities, infliximab therapy was instituted, which resulted in complete resolution of arthritis and skin lesions within 6 weeks of infliximab therapy. Discussion and Conclusion There is a lot of evidence supporting the use of TNF-a blockers in other seronegative spondyloarthropathies, such as psoriatic arthritis and AS, but there is only 1 previous case report of its use in Reiter9s syndrome. Although this use is intuitive, there is a theoretical potential as well as animal data suggesting its possible benefit. The successful use of infliximab in this patient with Reiter9s syndrome as well as rapid improvement in severe skin lesions and arthritis suggests a significant role of TNF-a in the pathogenesis of this disease. This is an encouraging preliminary result, and we suggest that the use of TNF-a blockers in Reiter9s syndrome should be further investigated.