Mixed cryoglobulinemia: demographic, clinical, and serologic features and survival in 231 patients

Ferri, Clodoveo; Sebastiani, Marco; Giuggioli, Dilia; Cazzato, Massimiliano; Longombardo, G.; Antonelli, Alessandro; Puccini, R; Michelassi, Claudio; Zignego, Anna Linda

doi:10.1016/j.semarthrit.2003.10.001

Cited by 502 publications

(515 citation statements)

References 83 publications

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“…In certain other systemic diseases associated with autoimmunity (e.g., systemic sclerosis), investigators distinguish between limited and diffuse disease subsets (37). In the case of systemic sclerosis, limited does not necessarily imply a lower degree of disease severity; rather, it refers to the extent of skin involvement, which is more circumscribed in that subset compared with the diffuse form.…”

Section: Discussionmentioning

confidence: 99%

Limited versus severe Wegener's granulomatosis: Baseline data on patients in the Wegener's granulomatosis etanercept trial

Stone¹

2003

Arthritis & Rheumatism

360

153

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Objective. To report baseline data on 180 patients with Wegener's granulomatosis (WG) enrolled in the WG Etanercept Trial (WGET), and to examine demographic and clinical differences between patients with limited disease and those with severe disease.Methods. Definitions of limited and severe disease were agreed upon by consensus of the investigators at a pretrial meeting and were incorporated into the protocol as a stratification criterion. These data were applied prospectively to the WGET patient cohort, based on clinical features and the intention to treat patients according to disease activity. Data related to disease onset, date of diagnosis, clinical features, antineutrophil cytoplasmic antibody assays, tissue biopsy findings, and other medical history were collected on a baseline medical history form. Physician-investigators from each center participated in the development of this form, and all were certified in its use prior to the start of the trial. Selected data on patients who were screened for the trial but were not enrolled were also collected.Results. Several significant differences between the limited and severe disease subsets were observed. Patients with limited disease were nearly a decade younger at disease onset compared with patients with severe disease. Thirty-three percent of patients with severe disease were women, compared with 58% of those with limited disease. Despite their younger age at symptom onset, patients with limited disease tended to have longer disease duration, a greater likelihood of experiencing exacerbation of previous disease following a period of remission, and a higher prevalence of destructive upper respiratory tract disorders at the time of enrollment (e.g., saddle-nose deformity). Patients with limited WG were less likely than those with severe disease to have antibodies to either proteinase 3 or myeloperoxidase. Patients with severe disease had a higher likelihood of previous thyroid disease, particularly either Graves' disease or Hashimoto thyroiditis, suggesting the possibility of different pathogenetic factors within these disease subsets. Other observed differences between these subsets, such as the greater frequency of alveolar hemorrhage in the severe disease group, were related to the a priori definitions of limited and severe disease.Conclusion. There are significant differences between patients with limited WG and those with severe WG with regard to sex, age, the likelihood of recurrent disease, the risk of damage in certain organ systems, and, possibly, etiologic factors. These differences (and perhaps other differences that are currently unrecognized) in patient subsets may have implications for mechanisms of pathogenesis, prognosis, response to treatment, and the design of future clinical investigations.

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Section: Discussionmentioning

confidence: 99%

Limited versus severe Wegener's granulomatosis: Baseline data on patients in the Wegener's granulomatosis etanercept trial

Stone¹

2003

Arthritis & Rheumatism

360

153

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“…All patients studied fulfilled the American College of Rheumatology (formerly, the American Rheumatism Association) criteria for the classification of SSc (19). Patients were grouped according to the 2-cutaneous subset classification (1,20); namely, patients were classified as having limited cutaneous SSc (lcSSc; sclerosis of both distal extremities, not the elbow and knees, with or without sclerosis of the neck and face) or diffuse cutaneous SSc (dcSSc; sclerosis of both distal and proximal extremities, with or without truncal involvement). Patients with overlap symptoms to other connective diseases were excluded from the study.…”

Section: Methodsmentioning

confidence: 99%

“…The patients included in the study had been evaluated for organ involvement at least 1 month before the blood sampling. Organ involvement was assessed according to previously described criteria (20). In particular, skin involvement was assessed by a modified Rodnan skin thickness score.…”

Section: Methodsmentioning

confidence: 99%

Circulating endothelial cells as a marker of ongoing vascular disease in systemic sclerosis

Papa

Colombo

Fracchiolla

et al. 2004

Arthritis & Rheumatism

136

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Results. Total and activated CEC counts were significantly higher in SSc patients compared with healthy controls and were positively correlated with the disease activity score. With respect to visceral involvement, significant correlation was observed between the CEC number and the severity of pulmonary hypertension. High levels of endothelial progenitors were observed in patients with SSc, and the counts were higher in the early stages of disease.

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“…46,47 Deposition of immune complexes in epineural vessels leading to vasculitis cause the ischemic damage to the nerves which is reflected as axonal damage on electrophysiological studies. 48 The symptoms are paresthesias, painful dysaesthesias, and myalgias.…”

Section: Nervous System Involvementmentioning

confidence: 99%

Hepatotropic Viral Infection Associated Systemic Vasculitides—Hepatitis B Virus Associated Polyarteritis Nodosa and Hepatitis C Virus Associated Cryoglobulinemic Vasculitis

Sharma

2013

Journal of Clinical and Experimental Hepatology

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Two hepatotropic viruses have been shown to have causal relationship with systemic vasculitis-hepatitis B with classical polyarteritis nodosa and hepatitis C with cryoglobulinemic vasculitis. The present paper provides an updated overview on the clinical presentations and management of these vasculitides.HBV associated PAN patients have higher weight loss, peripheral neuropathy, mononeuritis multiplex, abdominal pain, gastrointestinal manifestations requiring surgery, cardiomyopathy, orchitis, hypertension, and/or elevated transaminase levels. Microaneurysms are also more common in mesenteric artery. Skin manifestations, however are less common. These patients also have a severe disease as suggested by higher five factor score and higher BVAS. Though relapses are less common, mortality is higher in patients with HBV PAN as compared to non HBV PAN. Plasmapheresis has a role in treatment in clearing off immune complexes.The common clinical manifestations of HCV associated cryoglobulinemic vasculitis are skin lesions, peripheral neuropathy, glomerulonephritis, arthritis, and sicca symptoms. Though combination therapy comprising of pegylated interferon a and ribavirin is the first line of management, immunotherapy is needed for severe or life threatening manifestations. Recent randomized trials have shown the efficacy of rituximab in such situations. ( J CLIN EXP HEPATOL 2013;3:204-212)

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Mixed cryoglobulinemia: demographic, clinical, and serologic features and survival in 231 patients

Cited by 502 publications

References 83 publications

Limited versus severe Wegener's granulomatosis: Baseline data on patients in the Wegener's granulomatosis etanercept trial

Limited versus severe Wegener's granulomatosis: Baseline data on patients in the Wegener's granulomatosis etanercept trial

Circulating endothelial cells as a marker of ongoing vascular disease in systemic sclerosis

Hepatotropic Viral Infection Associated Systemic Vasculitides—Hepatitis B Virus Associated Polyarteritis Nodosa and Hepatitis C Virus Associated Cryoglobulinemic Vasculitis

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