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Arteropathies, Microcirculation and Vasculitis
Published in Mary N. Sheppard, Practical Cardiovascular Pathology, 2022
The term cryoglobulinaemia refers to the presence in the serum of one (monoclonal cryoimmunoglobulinaemia) or more (mixed cryoglobulinaemia) immunoglobulins, which precipitate at temperatures below 37°C and redissolve on rewarming. Circulating mixed cryoglobulins are often detected in many infectious and systemic disorders. It is characterized by leucocytoclastic vasculitis of small- and medium-sized vessels, and frequent multiple organ involvement due to cryoglobulin-containing immune complexes. Common signs and symptoms are a rash on the lower limbs, arthritis and nerve damage. Cryoglobulinaemia is classified into three types (I, II and III) on the basis of immunoglobulin composition. Predisposing conditions include lymphoproliferative disease, collagen disease and hepatitis C virus (HCV) infection. Cryoglobulinaemia generally leads to a systemic inflammatory syndrome characterized by fatigue, arthralgia, purpura, neuropathy and glomerulonephritis. The diagnosis of cryoglobulinaemia is based on the laboratory demonstration of serum cryoglobulins.
Cross-Reactive Idiotypes of Rheumatoid Factors in Arthritis and Related Diseases
Published in Thomas F. Kresina, Monoclonal Antibodies, Cytokines, and Arthritis, 2020
Pojen P. Chen, Dennis A. Carson
Although RFs were originally discovered in patients with RA, they have also been associated with some IgM paraproteins from patients with cryoglobulinemia and chronic lymphocytic leukemia (CLL) of B cell type (7-10). The cryoglobulins have been classified into three groups (9). Type I cryoglobulins consist of only monolconal Ig molecules and are found mainly in patients with lymphoproliferative diseases, such as multiple myeloma and Waldenstrom’s macroglobulinemia. Type II cryoglobulins are made of a monoclonal antibody together with polyclonal IgG. Type III cryoglobulins are composed of polyclonal antibodies and polyclonal Ig (or non-Ig) molecules. Based on their composition, type II and III cryoglobulins are often called “mixed cryoglobulins.” Most type II cryoglobulins contain monoclonal IgM RFs and polyclonal IgG and occur frequently in patients with Waldenstrom’s macroglobulinemia, chronic active hepatitis, or Sjogren’s syndrome (SS). In contrast, type III cryoglobulins are often associated with autoimmune conditions. Because of the common occurrence of monoclonal RFs from patients with the type II cryoglobulinemia, they have been used extensively in delineating the idiotypes and molecular genetics of human RFs.
Unexplained Fever In Hematologic Disorders Section 1. Benign Hematologic Disorders
Published in Benedict Isaac, Serge Kernbaum, Michael Burke, Unexplained Fever, 2019
Nonthrombocytopenic purpura can be seen in cryoglobulinemia, usually of the “mixed” type. The condition can be primary, or secondary to various collagen-vascular diseases or to infectious disease. Exposure to cold causes the precipitation of the cryoglobulins and can provoke Raynaud’s phenomenon, various skin lesions, and the hyperviscosity syndrome.
Current perspectives on the diagnosis, assessment, and management of vasculitic neuropathy
Published in Expert Review of Neurotherapeutics, 2022
Yuki Fukami, Haruki Koike, Masahisa Katsuno
Secondary systemic vasculitides are a heterogeneous group including vasculitis associated with infections, connective tissue diseases, malignancies, inflammatory bowel disease, hypocomplementemic urticarial vasculitis syndrome, and drug-induced vasculitis [5]. Vasculitic neuropathy can be associated with systemic rheumatic diseases. RA is complicated by rheumatoid vasculitis (RV) in 20–40% of patients [1,37]. Moreover, approximately 40% of patients with RV have sensory neuropathy, and up to 20% develop mixed symptoms of motor and sensory neuropathy [1]. In SLE, vasculitic mono- and multiple neuropathies tend to present acutely or subacutely with painful sensory and motor dysfunction [38]. It is estimated that 10–20% of patients with SLE have peripheral nervous system involvement [39], and mononeuritis multiplex occurs in 3% of 102 primary Sjögren’s syndrome patients [40]. According to a study involving 92 patients with neuropathy associated with Sjögren’s syndrome, 11 manifested mononeuritis multiplex and were presumed to have vasculitis [28]. Even if peripheral neuropathy is rare in sarcoidosis, epineural and perineural granulomas and granulomatous vasculitis can cause ischemic axonal degeneration and demyelination due to local pressure [41]. Cryoglobulinemia is a secondary vasculitis caused by deposition of circulating cryoglobulins, causing peripheral neuropathy in up to 60% of patients with mixed cryoglobulinemia syndrome, often associated with hepatitis C, hepatitis B, or human immunodeficiency virus infection [42].
Managing complications secondary to Waldenström’s macroglobulinemia
Published in Expert Review of Hematology, 2021
Ilias Pessach, Meletios A. Dimopoulos, Efstathios Kastritis
Cryoglobulins are serum proteins or protein complexes that precipitate at low temperatures. In type I cryoglobulinaemia, monoclonal IgM precipitates with 10% to 20% of patients with WM having positive test, but less than 5% being symptomatic because of these cryoglobulins [48]. Symptoms of cryoglobulinemia may include acrocyanosis, Raynaud’s phenomenon, palpable purpura, or glomerulonephritis and in view of these complications one should consider PLEX (through a heated circuit) followed by systemic treatment to achieve long-term control [49]. IgM flare may complicate therapy with anti-CD20 MoAbs and can aggravate symptoms, so that preemptive PLEX may be considered [50]. Ibrutinib may be a reasonable therapeutic option, PIs may also reduce IgM levels before initiation of anti-CD20 [51] and BR is another active 1st line treatment option [46,47].
Uncommon immune-mediated extrahepatic manifestations of HCV infection
Published in Expert Review of Clinical Immunology, 2018
Ciro Romano, Giovanna Cuomo, Roberta Ferrara, Andrea Del Mastro, Sergio Esposito, Ausilia Sellitto, Luigi Elio Adinolfi
Peripheral ulcerative keratitis (PUK) is known to be associated with rheumatoid arthritis for more than 50% of cases and with other rheumatic diseases (e.g. systemic lupus erythematosus, psoriatic arthritis, ANCA-associated vasculitis, and polyarteritis nodosa) in the remaining fraction of patients [63]. In a minority of HCV-related type II mixed cryoglobulinemia patients, PUK may complicate the course of disease [64,65]. The pathogenetic mechanism is supposed to be local precipitation of cryoglobulins, even in the absence of systemic involvement. Indeed, in the anterior segment of the eye, there is a temperature difference of 2°C cooler than the normal body temperature which may predispose cryoglobulins to precipitate just locally [65]. This may also explain why episodes of PUK (Figure 4) occur more frequently in the winter season in HCV-infected patients.