Vikas Majithia

American College of Rheumatology/Spondylitis Association of America/Spondyloarthritis Research and Treatment Network 2015 Recommendations for the Treatment of Ankylosing Spondylitis and Nonradiographic Axial Spondyloarthritis

To provide evidence‐based recommendations for the treatment of patients with ankylosing spondylitis (AS) and nonradiographic axial spondyloarthritis (SpA).

Weight Loss, the Obesity Paradox, and the Risk of Death in Rheumatoid Arthritis

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.

Cocaine use and its rheumatic manifestations: a case report and discussion

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.

Myasthenia gravis and systemic lupus erythematosus: truly associated or coincidental—two case reports and review of the literature

Systemic lupus erythematosus (SLE) andmyasthenia gravis (MG) are autoimmune disorders that have been reported to coexist [1–3]. These two diseases share many pathogenetic and epidemiological features. Several genetic, immunological, and environmental factors said to play a role in causation of these diseases are also common to both [2]. Despite these similarities, they are two distinct clinical syndromes [2]. The coexistence of SLE and MG is well documented, but there have been no controlled epidemiological studies to prove that a real association exists [2]. Here we report two cases of MG in patients with underlying SLE and review current literature.

Devic's syndrome as initial presentation of systemic lupus erythematosus.

Background:Devics 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 Devics 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.

American College of Rheumatology/Spondylitis Association of America/Spondyloarthritis Research and Treatment Network 2015 Recommendations for the Treatment of Ankylosing Spondylitis and Nonradiographic Axial Spondyloarthritis: ACR/SAA/Spartan Treatment Recommendations in AS

To provide evidence‐based recommendations for the treatment of patients with ankylosing spondylitis (AS) and nonradiographic axial spondyloarthritis (SpA).

Transverse myelitis, a rare neurological manifestation of mixed connective tissue disease--a case report and a review of literature.

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.

Nephrogenic Fibrosing Dermopathy, a New Mimicker of Systemic Sclerosis

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.

HLA DRB1*1503 allelic haplotype predominance and associated immunodysregulation in systemic lupus erythematosus

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.

Membranous (Class V) Renal Disease in Systemic Lupus Erythematosus May Be More Common Than Previously Reported: Results of a 6-Year Retrospective Analysis

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%. Conclusion: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.