Cutaneous small-vessel vasculitis (CSVV) is a general term for diseases that present pathological features of leukocytoclastic vasculitis, i.e., diseases with pathological perivascular neutrophilic infiltration and fibrinoid degeneration of the vascular walls (Fig. 11.1). Henoch-Schönlein purpura and urticarial vasculitis are included under a broad definition of CSVV; however, they are usually ...

Cryoglobulinaemia refers to circulating cryoglobulins. Cryoglobulins are immunoglobulins, which precipitate in temperatures below 37oC and dissolve upon rewarming [2]. Cryoglobu‐ linaemia is classified in 3 types based on clonality and immunoglobulin class. In particular, type I consists of monoclonal IgM or IgG immunoglobulin, type II is a mixture of monoclo‐ nal IgM and polyclonal IgG, while ...

The diagnosis and management of SVV remains one of the most challenging clinical scenarios encountered by a clinician. Careful attention to detail and a thorough knowledge of the specific disorders, their therapies, and complications thereof is required to optimally care for these patients. The recent completion of a number of randomized, controlled, multicenter clinical trials has greatly impr...

Antineutrophil cytoplasmic autoantibodies (ANCAs) directed to proteinase 3 (PR3-ANCA) or myeloperoxidase (MPO-ANCA) are strongly associated with the ANCA-associated vasculitides--Wegener's granulomatosis, microscopic polyangiitis, and Churg-Strauss syndrome. Clinical observations, including the efficacy of B-cell depletion via rituximab treatment, support--but do not prove--a pathogenic role fo...

Cutaneous vasculitis may present as a significant component of many systemic vasculitic syndromes such as rheumatoid vasculitis or anti-neutrophil cytoplasmic antibody (ANCA) associated primary vasculitic syndromes like microscopic polyangiits. Cutaneous vasculitis manifested most often as palpable purpura or infiltrated erythema indicating dermal small vessel vasculitis and less commonly as no...

Background: Sub-acute bacterial endocarditis (SBE) rarely presents with features of a small vessel vasculitis. Patients with SBE can also develop multiple serological abnormalities including ANCA. In this report, we present a case of infective endocarditis mimicked ANCA associated glomerulonephritis.Case presentation: A 57-year old male with a clinical picture of rapidly progressive renal failu...

The term vasculitis encompasses a number of distinct clinicopathologic disease entities, each of which is characterized pathologically by cellular inflammation and destruction of the blood vessel wall, and clinically by the types and locations of the affected vessels. While multiple classification schemes have been proposed to categorize and simplify the approach to these diseases, ultimately t...

Background: Wegener granulomatosis or granulomatosis with polyangiitis is a pauci-immune small vessel vasculitis which is usually associated with anti-neutrophil cytoplasmic antibodies (ANCA) mainly in old men. This small vessel vasculitis is usually characterized by necrotizing granulomatous inflammation with multiorgan involvement. Kidneys could be involved as the main and life-threatening co...

Granulomatosis with polyangiitis (GPA) is characterized by necrotizing granulomatous inflammation usually involving the upper and lower respiratory tracts and necrotizing vasculitis predominantly affecting small-sized vessels. Although categorized as a small vessel vasculitis, GPA can cause periaortitis and pericarditis. Physicians should recognize all categories of vasculitis can affect any si...