Journal of autoimmunity
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Journal of autoimmunity · Feb 2014
Review Case ReportsDiagnosis and classification of central nervous system vasculitis.
Central nervous system vasculitis is one of the foremost diagnostic challenges in rheumatology. It results in inflammation and destruction of the vasculature within the CNS. When vasculitis is confined to brain, meninges or spinal cord, it is referred to as primary angiitis of the CNS. ⋯ Progressive debilitating and unexplained neurological deficits, associated with abnormal cerebrospinal fluid is the typical picture of the disease. Biopsy of the brain remains the gold standard diagnostic test. The differential diagnosis of CNS vasculitis is highly diverse with a broad array of mimics at the clinical, radiographic and angiographic levels.
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Journal of autoimmunity · Feb 2014
ReviewThe classification and diagnostic criteria of ankylosing spondylitis.
Ankylosing spondylitis is the prototype of immune-mediated inflammatory rheumatic diseases grouped under the term spondyloarthritis (SpA). An early diagnosis has now become increasingly important because effective therapies are available and anti-TNF drugs are even more effective if used in early stages of the disease. In ankylosing spondylitis, the 1984 modified New York criteria have been used widely in clinical studies and daily practice but are not applicable in early disease when the characteristic radiographic signs of sacroiliitis are not visible but active sacroiliitis is readily detectable by magnetic resonance imaging (MRI). ⋯ New classification criteria for the wider group of SpA have been proposed by ASAS (Assessment of Spondylo Arthritis International Society); and the patients are sub-grouped into (1) a predominantly axial disease, termed axial SpA including AS and non-radiographic axial SpA; (2) peripheral SpA. The clinical course and disease process of non-radiographic axial spondyloarthritis remains unclear. However the development of the SpA criteria by ASAS particularly for axial SpA, is an important step for early diagnosis and better management of these patients.
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Journal of autoimmunity · Feb 2014
ReviewDiagnosis and classification of eosinophilic granulomatosis with polyangiitis (formerly named Churg-Strauss syndrome).
Recently, a group of experts in the field suggested to rename Churg-Strauss syndrome as eosinophilic granulomatosis with polyangiitis (EGPA). This condition, first described in 1951, is a rare small- and medium-sized-vessel vasculitis characterized by an almost constant association with asthma and eosinophilia, and, by the presence of anti-myeloperoxidase (MPO) antineutrophil cytoplasm antibodies (ANCA) in 30-38% of the patients. Vasculitis typically develops in a previously asthmatic and eosinophilic middle-aged patient. ⋯ This observation led to the hypothesis that EGPA might be divided into different clinical and pathophysiological subtypes, which could be managed better with more specifically adapted therapies. For now, EGPA treatment still relies mainly on corticosteroids and, when necessary for patients with poorer prognoses, combined immunosuppressant drugs, especially cyclophosphamide. Overall survival of EGPA patients is good, despite not uncommon relapses.
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Journal of autoimmunity · Feb 2014
ReviewThe diagnosis of multiple sclerosis and the various related demyelinating syndromes: a critical review.
Multiple sclerosis (MS), is a chronic disease of the central nervous system (CNS) characterized by loss of motor and sensory function, that results from immune-mediated inflammation, demyelination and subsequent axonal damage. MS is one of the most common causes of neurological disability in young adults. Several variants of MS (and CNS demyelinating syndromes in general) have been nowadays defined in an effort to increase the diagnostic accuracy, to identify the unique immunopathogenic profile and to tailor treatment in each individual patient. ⋯ The unique clinical and neuroradiological features, along with the immunological biomarkers help to distinguish these cases from classical MS. The use of such immunological and imaging biomarkers, will not only improve the accuracy of diagnosis but also contribute to the identification of the patients with CIS or RIS who, are at greater risk for disability progression (worse prognosis) or, on the contrary, will have a more benign course. This review summarizes in a critical way, the diagnostic criteria (historical and updated) and the definitions/characteristics of MS of the various variants/subtypes of CNS demyelinating syndromes.
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Journal of autoimmunity · Oct 2013
ReviewClinical significance of autoantibodies in autoimmune hepatitis.
The accurate diagnosis and classification of autoimmune hepatitis (AIH) rely upon the detection of characteristic autoantibodies. Positivity for anti-nuclear (ANA) and/or anti-smooth muscle (SMA) autoantibodies defines AIH type 1 (AIH-1), whereas anti-liver kidney microsomal type 1 (anti-LKM1) and/or anti-liver cytosol type 1 (anti-LC1) define AIH type 2 (AIH-2). ANA and SMA, and less commonly anti-LKM1, have also been detected in de-novo autoimmune hepatitis developing after liver transplantation, a condition that may affect patients transplanted for non-autoimmune liver disease. ⋯ These associations highlight the necessity for simple and prompt diagnostic autoantibody testing, and the requirement for the accurate interpretation of the results of the tests in the clinical context. Fine-mapping of antigenic autoantibody targets has facilitated the development of rapid molecular assays that have the potential to revolutionise the field if properly standardised and when used in combination with classical immunofluorescence. Despite their diagnostic significance, the pathogenic role of the various autoantibodies and the mechanisms by which they can potentially inflict damage onto the liver cell remain a topic for further research.