Mixed-Cryoglobulinemia Associated with Cutaneous Vasculitis and Pulmonary Symptoms.

Konishi, Masahiro; Ohosone, Yasuo; Matsumura, Mai; Oyamada, Yoshitaka; Yamaguchi, Kazuhiro; Kawahara, Yoshie; Mimori, Tsuneyo; Ikeda, Yasuo

doi:10.2169/internalmedicine.36.62

Cited by 12 publications

(3 citation statements)

References 10 publications

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“…Other authors have reported vasculitis in 30% of both primary and secondary SS patients [13]. Palpable purpura is also found in SS patients [14] with biopsies showing leukocytoclastic vasculitis [12] and may be associated with central nervous system involvement [15] or pulmonary involvement [16].…”

Section: Manifestations and Differential Diagnosis In The Pediatric P...mentioning

confidence: 99%

Extraglandular Manifestations of Sjögren’s Syndrome (SS): Dermatologic, Arthritic, Endocrine, Pulmonary, Cardiovascular, Gastroenterology, Renal, Urology, and Gynecologic Manifestations

Fox

2011

Sjögren’s Syndrome

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Primary Sjögren's syndrome (1 • SS) is an autoimmune disorder characterized by dry eyes (keratoconjunctivitis sicca) and dry mouth due to lymphocytic infiltrates of lacrimal and salivary glands. However, SS also affects many extraglandular systems. In SS patients, the pattern of extraglandular manifestations may have a close similarity with the vasculitic features seen in SLE patients that are mediated by immune complexes and complement. However, SS patients also have an increased frequency of lymphocytic infiltration into extraglandular tissues, as might be expected by their increased frequency of lymphoma in comparison to SLE patients. For example, SS patients need to be evaluated for interstitial nephritis (in contrast to the glomerulonephritis of SLE) or interstitial pneumonitis (in comparison to pleurisy of SLE). This chapter will focus on the clinical extraglandular manifestations of primary SS that are not specifically covered in other chapters. These extraglandular manifestations have led to a recently introduced "disease activity" and "organ damage index." The recognition of these extraglandular manifestations is important since they have prognostic and therapeutic implications. The differential diagnosis of these extraglandular manifestations includes overlapping features with other autoimmune diseases (particularly systemic lupus erythematosus (SLE), scleroderma, dermatomyositis, celiac sprue, and small-and medium-sized vessel vasculitis), infectious diseases that mimic autoimmune disease (particularly hepatitis C, HIV, syphilis, tuberculosis), and predisposition to drug toxicities that may involve extraglandular organs (particularly skin rashes, nephritis, pneumonitis, myositis, and hematopoietic abnormalities). R.I. Fox ( )

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Section: Manifestations and Differential Diagnosis In The Pediatric P...mentioning

confidence: 99%

Extraglandular Manifestations of Sjögren’s Syndrome (SS): Dermatologic, Arthritic, Endocrine, Pulmonary, Cardiovascular, Gastroenterology, Renal, Urology, and Gynecologic Manifestations

Fox

2011

Sjögren’s Syndrome

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“…(Bernacchi et al, 2004). Palpable purpura is also found in SS patients (Alexander and Provost, 1987) with biopsies showing leukocytoclastic vasculitis (Ramos-Casals et al, 1998) and may be associated with central nervous system involvement or pulmonary involvement (Konishi et al, 1997).…”

Section: Cryoglobulinemia Is Divided Into Three Clinical Subsets: Typmentioning

confidence: 99%

Sjögren’s Syndrome

Fox

Fox²

2011

Autoimmune Diseases of the Skin

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“…Cryoglobulinemia is associated with various overlying diseases and involves a variety of symptoms such as purpura, arthralgias, neuropathies, and hepathic and renal involvement. 2,3 Severe vasculitic complication, such as digital necrosis, is very rare and predominantly affects small vessels. Arteritis of small-and medium-sized vessels is present in a significant number of patients with systemic lupus erythematosus.…”

Section: Introductionmentioning

confidence: 99%

Type III Mixed Cryoglobulinemia Associated with Digital Necrotic Ulcer Successfully Treated with Intermittent Intravenous Pulse Cyclophosphamide

2005

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Cryoglobulinemic vasculitis is an immune complex-mediated vasculitis predominantly affecting small vessels. The authors report an 18-year-old woman with painful digital necrotic ulcer due to type III cryoglobulinemic vasculitis on the basis of systemic lupus erythematosus. Serum protein electrophoresis and immunoelectrophoresis demonstrated a polyclonal peak of the immunoglobulin Glambda (IgGlambda) and IgMlambda type. The patient was successfully treated with intermittent intravenous pulse cyclophosphamide.

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Mixed-Cryoglobulinemia Associated with Cutaneous Vasculitis and Pulmonary Symptoms.

Cited by 12 publications

References 10 publications

Extraglandular Manifestations of Sjögren’s Syndrome (SS): Dermatologic, Arthritic, Endocrine, Pulmonary, Cardiovascular, Gastroenterology, Renal, Urology, and Gynecologic Manifestations

Extraglandular Manifestations of Sjögren’s Syndrome (SS): Dermatologic, Arthritic, Endocrine, Pulmonary, Cardiovascular, Gastroenterology, Renal, Urology, and Gynecologic Manifestations

Sjögren’s Syndrome

Type III Mixed Cryoglobulinemia Associated with Digital Necrotic Ulcer Successfully Treated with Intermittent Intravenous Pulse Cyclophosphamide

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