Circulating 20S proteasome levels in patients with mixed connective tissue disease and systemic lupus erythematosus.

The associations of circulating 20S proteasomes (c20S) with clinical and serologic disease indices in patients with systemic lupus erythematosus (SLE) and mixed connective tissue disease (MCTD) are unknown. We present the initial report that c20S levels are elevated in MCTD and correlate with clinically relevant changes in disease activity in SLE and MCTD.

Immune pathogenesis of Mixed Connective Tissue Disease: a short analytical review.

Mixed Connective Tissue Disease (MCTD) was first described 35 years ago by Gordon C. Sharp and his colleagues. In the ensuing decades, a clearer understanding of the clinical and serologic features of MCTD has emerged.

Clinical and immunologic manifestations of mixed connective tissue disease in a Miami population compared to a Midwestern US Caucasian population.

Objective: A cross-sectional study of mixed connective tissue disease (MCTD) was performed to determine if there were identifiable differences in the clinical expression of MCTD associated with race or ethnicity.

Methods: Miami, Florida, and Midwestern US (Missouri) Caucasian MCTD cohorts were studied. Clinical and laboratory features of the 2 MCTD cohorts were compared.

The incidence and prevalence of neuropsychiatric syndromes in pediatric onset systemic lupus erythematosus.

Objective: To determine the incidence and prevalence of neuropsychiatric systemic lupus erythematosus (NPSLE) and glomerulonephritis in ethnically diverse pediatric onset SLE inpatient and outpatient populations.

Methods: Seventy-five pediatric onset patients with SLE including Native American, Asian, Black, Spanish-American, and Caucasian subjects were evaluated prospectively and cross sectionally. During the 6 year study, 55 patients became inpatients.

Neuropsychiatric systemic lupus erythematosus presenting as amyotrophic lateral sclerosis.

A 61-year-old woman with a history of photosensitive dermatitis and recurrent mouth ulcers presented with progressive weakness typical of amyotrophic lateral sclerosis (ALS), and subsequently underwent extensive neurologic and rheumatologic testing. We investigated whether ALS-like motor neuron disease associated with a positive antinuclear antibody (ANA) is really ALS or rather neuropsychiatric systemic lupus erythematosus (NPSLE). On neurologic evaluation, she had prominent bulbar involvement with dysarthria and dysphagia associated with profound lingual fasciculations and a denervating pattern on electromyogram.