Abstract
Immune axonal neuropathies are a particular group of immune-mediated neuropathies that occasionally accompany systemic autoimmune rheumatic diseases such as connective tissue dissorders and primary systemic vasculitides. Apart from vasculitis of vasa nervorum, various other mechanisms are involved in their pathogenesis, with possible therapeutic implications. Immune axonal neuropathies have highly heterogeneous clinical presentation and course, ranging from mild chronic distal sensorimotor polyneuropathy to severe subacute mononeuritis multiplex with rapid progression and constitutional symptoms such as fever, malaise, weight loss and night sweats, underpinning a vasculitic process. Sensory neuronopathy (ganglionopathy), small fiber neuropathy (sensory and/or autonomic), axonal variants of Guillain-Barré syndrome and cranial neuropathies have also been reported. In contrast to demyelinating neuropathies, immune axonal neuropathies show absent or reduced nerve amplitudes with normal latencies and conduction velocities on nerve conduction studies. Diagnosis and initiation of treatment are often delayed, leading to accumulating disability. Considering the lack of validated diagnostic criteria and evidence-based treatment protocols for immune axonal neuropathies, this review offers a comprehensive perspective on etiopathogenesis, clinical and paraclinical findings as well as therapy guidance for assisting the clinician in approaching these patients. High quality clinical research is required in order to provide indications and follow up rules for treatment in immune axonal neuropathies related to systemic autoimmune rheumatic diseases.
Highlights
Autoimmune diseases are a broad group of conditions characterized by chronic activation of the immune system that eventually leads to tissue inflammation and damage (Doria et al, 2012)
We aim to review immune axonal neuropathies associated with systemic autoimmune rheumatic diseases (SARDs), with a special focus on vasculitic neuropathies encountered in systemic vasculitides, both primary and secondary to other SARDs
A peripheral nerve disorder is suspected based on history and physical examination, paraclinical tests are essential in making the final diagnosis of immunemediated axonal neuropathies
Summary
Autoimmune diseases are a broad group of conditions characterized by chronic activation of the immune system that eventually leads to tissue inflammation and damage (Doria et al, 2012). According to the type of the vessels involved, systemic vasculitides are further classified into predominantly large vessel vasculitides (e.g., giant cell arteritis), predominantly medium vessel vasculitides (e.g., polyarteritis nodosa (PAN)) and predominantly small vessel vasculitides (e.g., antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides and cryoglobulinemia) (Jennette et al, 2012) Another classification identifies primary systemic vasculitides (e.g., ANCA-associated vasculitides) and secondary systemic vasculitides that occur in the setting of other SARDs (e.g., lupus vasculitis, rheumatoid vasculitis, sarcoid vasculitis), various infections, drugs, malignancies, inflammatory bowel disease and hypocomplementemic urticarial vasculitis syndrome (Collins et al, 2010; Sampaio et al, 2011; Collins, 2012; Jennette et al, 2012)
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