Connective tissue diseases are characterized by different organ disorders due to loss of immune system tolerance to autoantigens. Peripheral neuropathy is one of the features of these diseases with variable frequency; it is more prevalent in Sjögren syndrome. Peripheral neuropathy is often seen in the course of the disease. Nonetheless, it may be also a presenting sign or the unique feature of immune system dysfunction. Neuropathies in connective tissue diseases are related mainly to vasculitic disorder. It requires prompt diagnosis and treatment to improve its outcome. Peripheral neuropathy in connective tissue diseases could be multifocal and asymmetric, or confluent and symmetrical. This chapter reviews the clinical, diagnostic and therapeutic features of neuropathies associated with the common diffuse connective tissue diseases.
Part of the book: Demystifying Polyneuropathy
Antineutrophil cytoplasmic antibodies (ANCA)-associated diseases are necrotizing systemic vasculitides that affect small blood vessels (arterioles, capillaries and venules). This entity represents three main systemic vasculitides: granulomatosis with polyangiitis (GPA; formerly Wegener’s granulomatosis), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA; formerly known as Churg-Strauss’ syndrome). Their clinical manifestations are polymorphous, being the most frequent respiratory, oto-laryngo-pharyngeal and renal involvement. Peripheral neuropathy (PN) is reported in almost 50% of the patients. The aim of this chapter is to discuss the prevalence, clinical presentation, treatment and prognosis of PN in ANCA-associated vasculitis.
Part of the book: Demyelination Disorders
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) constitute a group of necrotizing systemic vasculitis with preferential involvement of small- to medium-sized vessels. None treated; they are considered as a life-threatening illness by their renal, cardiac and neurologic damages. Therefore, treatment is usually aggressive, with high-dose corticosteroid therapy combined with immunosuppressive drugs in the major part of cases. New biologic drugs have been introduced such as rituximab. In this chapter, we will present the update and recent advances in the treatment of AAV.
Part of the book: Pericarditis