A Case Report of NMO Transverse Myelitis.

BACKGROUND: Transverse myelitis is considered one of the cardinal features of neuromyelitis optica spectrum disorder (NMOSD), an immune-mediated inflammatory condition of the CNS characterized by severe, immune-mediated demyelination and axonal damage predominantly targeting optic nerves and spinal cord. We describe a case in which a diagnosis of NMOSD was established, associated with West Nile Virus (WNV) infection. CASE PRESENTATION: A healthy 18-year-old female presented with intractable hiccups and rapidly progressing paraparesis. MRI demonstrated T2 edema extending from the medulla to the conus, consistent with longitudinally extensive transverse myelitis. Serum and CSF Aquaporin-4 IgG (AQP4) were both positive with high titers. In conjunction with antiviral therapy, immunomodulatory treatment was initiated using pulse methylprednisolone, plasmapheresis and Rituximab. A month and a half after admission, the patient was fully ambulatory with no residual symptoms. On her rheumatology follow-up visit, West Nile Virus-specific IgM in CSF was found to be positive from the patient's initial presentation. CONCLUSION: We propose that West Nile Virus may have been the autoimmune trigger to the patient's development of NMOSD, highlighting the importance of evaluating viral triggers in autoimmune diseases.

Leprosy, the Great Imitator of Rheumatic Diseases: A Case Study.

A 68-year-old Hispanic man was referred to our center for cutaneous vasculitis of the lower extremities, diagnosed via skin biopsy. He had a 10-year history of erythematous plaques complicated by persistent, non-healing ulcers previously treated with prednisone and hydroxychloroquine. Laboratory testing was significant for positive U1-ribonucleoprotein antibody, antinuclear antibody human epithelial-2, and an elevated erythrocyte sedimentation rate. A repeat skin biopsy revealed nonspecific ulcerations. The patient was diagnosed with a mixed connective tissue disease with features of scleroderma. Mycophenolate was initiated, and prednisone was tapered. After two years of relapsing ulcerations on his lower extremities, a third skin punch biopsy showed dermal granulomas with numerous acid-fast organisms, and a polymerase chain reaction identified Mycobacterium lepromatosis, indicating polar lepromatous leprosy with an erythema nodosum leprosum reaction. After three months of minocycline and rifampin therapy, his lower extremity ulcerations and erythema resolved. Our case highlights the variable and elusive nature of this disease, which can mimic many systemic rheumatologic conditions.

Adalimumab-Induced Lupus Serositis: A Case Report and Review of the Literature.

Tumor necrosis factor-alpha (TNF-alpha) antagonist use is prevalent for the treatment of autoimmune diseases, including psoriasis, ankylosing spondylitis, and rheumatoid arthritis. Since the onset of its use over the last couple of decades, there have been increasing reports of drug-induced antibodies and antitumor necrosis factor-alpha-induced lupus (ATIL). Herein, we present a case of pericarditis induced by tumor necrosis factor-alpha antagonist, adalimumab. A 61-year-old male with psoriatic arthritis treated with adalimumab injections for five years presented with dyspnea, chest tightness, and three-pillow orthopnea. Echocardiogram showed moderate pericardial effusion with early signs of tamponade. Adalimumab was discontinued. He was started on colchicine and steroids for a high suspicion of drug-induced serositis. With the increased use of tumor necrosis factor-alpha antagonists, adverse reactions such as ATIL will become more common. Such cases need to be reported to spread awareness of this possible complication and avoid any delay in treatment and care.

Longitudinal Trends of Hospitalizations for Giant Cell Arteritis: A 21-Year Longitudinal National Population-Based Study.

Background Long-term longitudinal studies on giant cell arteritis (GCA) hospitalizations are limited. Here we aim to fill gaps in knowledge by analyzing longitudinal trends of GCA hospitalizations over the last two decades in the United States (U.S.). Materials and methods We performed a 21-year longitudinal trend analysis of GCA hospitalizations using data obtained from the National Inpatient Sample (NIS) database between 1998 and 2018. Using the NIS database, we searched for hospitalizations for patients aged ≥ 50 years with a principal diagnosis of GCA using ICD billing codes. The principal diagnosis was the main reason for hospitalization. We used all hospitalizations in patients without GCA aged ≥50 years as the control population. Multivariable logistic and linear regression analysis was utilized to calculate the adjusted p-trend for outcomes of interest. Results The incidence of GCA hospitalization remained stable at about one per 100,000 U.S. persons throughout the study period. There was no statistically significant change in the inpatient mortality for the GCA group during the study period (adjusted p-trend=0.111). In comparison, inpatient mortality reduced from 4.4% to 3.1% from 1998 to 2018 (adjusted p-trend <0.0001) in the control group. The proportion of whites reduced, while the proportion of racial minorities increased over time in both the GCA and control groups. Conclusion The non-GCA control population saw significant reductions in mortality over time, but unfortunately, the GCA group did not see such improvements. More research into additional treatment modalities for inpatient GCA management may help improve mortality.

Analysis of Emergency Department Visits by Patients With Giant Cell Arteritis: A National Population-Based Study.

Background There is scarcity of national level data on the reasons for Emergency Department (ED) presentation among patients with Giant cell arteritis (GCA) in the United States. This study aims to outline the most common reasons for ED presentation among these patients, and the baseline characteristics and outcomes of ED visits principally for GCA.  Materials and methods We obtained data from the Nationwide Emergency Department Sample (NEDS) 2018 database. Each ED visit in the NEDS has a principal diagnosis (the main reason for the visit) and can have up to 34 other secondary diagnoses. We searched for ED visits for patients aged ≥50 with any diagnosis of GCA using ICD-10 codes. The most common principal discharge diagnoses were divided into organ systems, and specific principal discharge diagnoses were recorded for ED visits among patients with GCA in descending order of frequency. We then outlined baseline characteristics and outcomes of ED visits with a principal diagnosis of GCA. Results There were 20,886 ED visits for patients with GCA in 2018. Infections, as well as rheumatologic and cardiovascular disease were the most common reasons for ED presentation, and GCA was the most common specific principal discharge diagnosis for ED visits. There were 3888 ED visits with a principal diagnosis of GCA. These patients were predominantly elderly females, admitted, Medicare insured, with minimal comorbidity burden, and presented to metropolitan teaching hospitals in the south. Conclusion GCA patients are most likely to present to the ED due to their underlying GCA. Infections and CV are also common reasons for presentation to the ED.

Healthcare disparities in rheumatology: the role of education at a global level.

All fields of medicine are victim to health inequity worldwide, including rheumatology. While the health system is a key proponent to health access for all, other social determinants of health also impact world health. We describe herein the current state of global healthcare disparities in rheumatology and attempts at optimizing graduate medical education and resources for optimized healthcare, international research collaborations and a future of universal health equity. We performed a comprehensive search through Pubmed using the following keywords: healthcare disparities, medical education, access to care, community health.Key Points• Healthcare disparities are ubiquitous globally, including the field of rheumatology.• The heterogeneity of global healthcare disparities emphasizes the importance of addressing unmet needs at a regional level.• A standardized approach to incorporating healthcare disparities education in the medical field is lacking. Intervening at this level provides a foundation of increasing provider awareness of regional healthcare disparities so as to establish a framework of addressing such disparities in a culturally competent manner.

Randomised prospective trial to assess the clinical utility of multianalyte assay panel with complement activation products for the diagnosis of SLE.

OBJECTIVE: We compared the physician-assessed diagnostic likelihood of SLE resulting from standard diagnosis laboratory testing (SDLT) to that resulting from multianalyte assay panel (MAP) with cell-bound complement activation products (MAP/CB-CAPs), which reports a two-tiered index test result having 80% sensitivity and 86% specificity for SLE. METHODS: Patients (n=145) with a history of positive antinuclear antibody status were evaluated clinically by rheumatologists and randomised to SDLT arm (tests ordered at the discretion of the rheumatologists) or to MAP/CB-CAPs testing arm. The primary endpoint was based on the change in the physician likelihood of SLE on a five-point Likert scale collected before and after testing. Changes in pharmacological treatment based on laboratory results were assessed in both arms. Statistical analysis consisted of Wilcoxon and Fisher's exact tests. RESULTS: At enrolment, patients randomised to SDLT (n=73, age=48±2 years, 94% females) and MAP/CB-CAPs testing arms (n=72, 50±2 years, 93% females) presented with similar pretest likelihood of SLE (1.42±0.06 vs 1.46±0.06 points, respectively; p=0.68). Post-test likelihood of SLE resulting from randomisation in the MAP/CB-CAPs testing arm was significantly lower than that resulting from randomisation to SDLT arm on review of test results (-0.44±0.10 points vs -0.19±0.07 points) and at the 12-week follow-up visit (-0.61±0.10 points vs -0.31±0.10 points) (p<0.05). Among patients randomised to the MAP/CB-CAPs testing arm, two-tiered positive test results associated significantly with initiation of prednisone (p=0.034). CONCLUSION: Our data suggest that MAP/CB-CAPs testing has clinical utility in facilitating SLE diagnosis and treatment decisions.

Chronic graft-versus-host disease presenting as eosinophilic fasciitis: therapeutic challenges and an additional case.

Chronic graft-versus-host disease (cGVHD) is one of the main late complications of allogeneic hematopoietic stem cell transplant and a major contributor to the mortality and morbidity in surviving recipients. Skin is the most common involved organ in cGVHD and may mimic a wide spectrum of dermatological conditions in its clinical and histopathologic manifestations. Some of the commonly simulated diseases are scleroderma, morphea, and lichen sclerosus. Chronic GVHD simulating eosinophilic fasciitis (EF) is relatively rare, frequently presenting with skin induration, a typical "peau d'orange" appearance, peripheral blood eosinophilia, myalgia, arthralgia, and arthritis leading to joint contractures in severe cases.Diagnosis is based on clinical manifestations and histopathology. Treatment is challenging because most cases are refractory to first-line therapy of glucocorticoids and calcineurin inhibitors (CNIs), and there is no standard second-line therapy.We report a comprehensive review of literature on all reported cases of CGVHD presenting as EF. We also describe an additional interesting case of cGVHD presenting as EF that was resistant to traditional therapy of high-dose glucocorticoids and cyclosporin A, but showed complete resolution of skin manifestations after addition of imatinib.Chronic GVHD presenting as EF is a rare variant of sclerodermatous cGVHD. Diagnosis is difficult, and treatment of cGVHD mimicking EF remains a therapeutic challenge because of obscure pathogenesis and poor response to traditional immunosuppressive medications. Emerging insights into the pathogenesis of cGVHD have resulted in the development of novel targeted therapies, which may improve outcomes and should be attempted in this subset of the disease. Larger studies are warranted to substantiate these preliminary findings.

Review of current therapies for secondary hypertrophic pulmonary osteoarthropathy.

Hypertrophic osteoarthropathy (HOA) is a disabling condition that may occur secondarily to primary lung cancer. It is characterized by digital clubbing, arthralgia/arthritis, and periostosis of the tubular bones. The pain associated with HOA can be disabling and often refractory to conventional analgesics. We performed a comprehensive review of the literature using the PubMed database on treatment modalities available for HOA. We found 52 relevant articles-40 case reports, six case series, two review papers, and four combined case series and review papers. There were no randomized controlled trials reported. We then classified treatments used for HOA into two categories: (1) treatment of primary cause (i.e., resection of tumor, chemotherapy, radiotherapy, treatment of infection, etc.) and (2) symptomatic treatments (i.e., bisphosphonates, octreotide, NSAIDs, vagotomy, etc.). Subsequently, we summarized the main findings for each treatment. Although the clinical diagnosis of HOA has existed for over 100 years, the pathogenesis mechanism has not yet been elucidated, and treatment options for this condition remain experimental. Primary treatment is the most widely reported modality to be efficacious. In cases which primary therapy is not possible, several symptomatic treatment modalities are suggested, with various degree of success. Further research is needed to clarify the pathophysiological mechanism of HOA as to appropriately direct therapy.

Sudden hearing loss as the presenting symptom of systemic sclerosis.

OBJECTIVES: Sudden sensorineural hearing loss (SSHNL) is an emergency in otolaryngology. In most cases, the exact cause cannot be identified, but different immunologic disorders and microvascular events have been suggested to play a role in its pathogenesis. Sudden sensorineural hearing loss can be caused by collagen vascular disorders, but it has rarely been reported as the presenting symptom of these diseases. This case presented with bilateral hearing loss and was finally diagnosed as a systemic sclerosis (SSc) patient. This is the first case of SSc that presented with SSNHL as an initial symptom. PATIENT: A 65-year-old man presented with bilateral hearing loss. He also complained of generalized fatigue and intermittent paresthesia. Brain imaging and blood tests were negative except for antinuclear antibodies. Administration of high-dose prednisone did not make any improvement. After extensive workup, follow-up, and referral to rheumatologist, the diagnosis of SSc was made. The patient's hearing improved after receiving intravenous immunoglobulin along with other symptoms of the disease. CONCLUSION: This case illustrates the importance of follow-up and appropriate of SSNHL patients with other systemic symptoms.