Elevated Cerebral Spinal Fluid Cytokine Levels in Boys with Cerebral Adrenoleukodystrophy Correlates with MRI Severity

Background: X-linked adrenoleukodystrophy (ALD) is a metabolic, peroxisomal disease that results from a mutation in the ABCD1 gene. The most severe course of ALD progression is the cerebral inflammatory and demyelinating form of the disease, cALD. To date there is very little information on the cytokine mediators in the cerebral spinal fluid (CSF) of these boys. Methodology/Principal Findings: Measurement of 23 different cytokines was performed on CSF and serum of boys with cerebral ALD and patients without ALD. Significant elevations in CSF IL-8 (29.362.2 vs 12.861.1 pg/ml, p = 0.0001), IL-1ra (166630 vs 8.666.5 pg/ml, p = 0.005), MCP-1 (610647 vs 328634 pg/ml, p = 0.002), and MIP-1b (14.261.3 vs 2.061.4 pg/ml, p,0.0001) were found in boys with cALD versus the control group. The only serum cytokine showing an elevation in the ALD group was SDF-1 (21246155 vs 11756125 pg/ml, p = 0.0001). The CSF cytokines of IL-8 and MCP-1b correlated with the Loes MRI severity score (p = 0.04 and p = 0.008 respectively), as well as the serum SDF-1 level (p = 0.002). Finally, CSF total protein was also significantly elevated in boys with cALD and correlated with both IL-8, MCP-1b (p = 0.0001 for both), as well as Loes MRI severity score (p = 0.0007). Conclusions/Significance: IL-8, IL-1ra, MCP-1, MIP-1b and CSF total protein were significantly elevated in patients with cALD; IL-8, MCP-1b, and CSF total protein levels correlated with disease severity determined by MRI. This is the largest repor

Autoimmune gastrointestinal complications in patients with Systemic Lupus Erythematosus: case series and literature review

The association of systemic lupus erythematosus (SLE) with gastrointestinal autoimmune diseases is rare, but has been described in the literature, mostly as case reports. However, some of these diseases may be very severe, thus a correct and early diagnosis with appropriate management are fundamental. We have analysed our data from the SLE patient cohort at University College Hospital London, established in 1978, identifying those patients with an associated autoimmune gastrointestinal disease. We have also undertaken a review of the literature describing the major autoimmune gastrointestinal pathologies which may be coincident with SLE, focusing on the incidence, clinical and laboratory (particularly antibody) findings, common aetiopathogenesis and complications

A link between irritable bowel syndrome and fibromyalgia may be related to findings on lactulose breath testing

Objective: To compare the prevalence and test results for bacterial overgrowth between IBS and fibromyalgia. Methods: Subjects with independent fibromyalgia and IBS were compared with controls in a double blind study. Participants completed a questionnaire, and a lactulose hydrogen breath test was used to determine the presence of SIBO. The prevalence of an abnormal breath test was compared between study participants. Hydrogen production on the breath test was compared between subjects with IBS and fibromyalgia. The somatic pain visual analogue score of subjects with fibromyalgia was compared with their degree of hydrogen production. Results: 3/15 (20%) controls had an abnormal breath test compared with 93/111 (84%) subjects with IBS (p<0.01) and 42/42 (100%) with fibromyalgia (p<0.0001 v controls, p<0.05 v IBS). Subjects with fibromyalgia had higher hydrogen profiles (p<0.01), peak hydrogen (p<0.0001), and area under the curve (p<0.01) than subjects with IBS. This was not dependent on the higher prevalence of an abnormal breath test. The degree of somatic pain in fibromyalgia correlated significantly with the hydrogen level seen on the breath test (r = 0.42, p<0.01). Conclusions: An abnormal lactulose breath test is more common in fibromyalgia than IBS. In contrast with IBS, the degree of abnormality on breath test is greater in subjects with fibromyalgia and correlates with somatic pain