Mixed cryoglobulinemia: Clinical aspects and long-term follow-up of 40 patients
Identifieur interne : 003981 ( Istex/Corpus ); précédent : 003980; suivant : 003982Mixed cryoglobulinemia: Clinical aspects and long-term follow-up of 40 patients
Auteurs : Peter D. Gorevic ; Hannah Jane Kassab ; Yoram Levo ; Robert Kohn ; Martin Meltzer ; Philip Prose ; Edward C. FranklinSource :
- The American Journal of Medicine [ 0002-9343 ] ; 1980.
Abstract
The clinical course of 40 patients with significant quantities of mixed cryoglobulins, but without lymphoproliferative, collagen-vascular or chronic infectious diseases, is presented. These cases comprise 51.3 percent of all mixed and 31.7 percent of all types of cryoglobulins evaluated by us over the period 1960–1978. A characteristic clinical syndrome, consisting of recurrent palpable purpura (100 percent), polyarthralgias (72.5 percent) and renal disease (55 percent), was seen. Biopsy specimens of skin lesions showed cutaneous vasculitis, and half had immune reactants in vessel walls. Seventy percent of patients had evidence of hepatic dysfunction, often subclinical, and more than 60 percent of those tested had serologic evidence of prior infection with hepatitis B virus. Hepatic lesions ranged from minimal triaditis to chronic active hepatitis and/or cirrhosis. All 22 patients in whom clinical renal disease developed had significant proteinuria; 63.6 percent had diastolic hypertension, 77.3 percent edema, 45.5 percent renal failure and 22.7 percent were nephrotic. Glomerular disease associated with deposition of immunoglobulin G, immunoglobulin M and complement, often with coexistent renal arteritis, was confirmed pathlologically in 15 cases.All cryoglobulins had rheumatoid factor activity and consisted of IgM and polyclonal IgG; five also contained IgA. Thirteen had a monoclonal IgM kappa component. Serum protein electrophoresis was unremarkable or showed diffuse hyperglobulinemia. Striking depression of early complement components was noted but did not correlate well with the cryoprotein concentration, renal involvement or clinical course.Follow-up for periods up to 21 years from onset of symptoms revealed that renal involvement has a deleterious effect on prognosis. Postmortem examinations of nine patients demonstrated widespread vasculitis in addition to renal involvement. Preterminal infection was found in eight.
Url:
DOI: 10.1016/0002-9343(80)90390-3
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<front><div type="abstract" xml:lang="en">The clinical course of 40 patients with significant quantities of mixed cryoglobulins, but without lymphoproliferative, collagen-vascular or chronic infectious diseases, is presented. These cases comprise 51.3 percent of all mixed and 31.7 percent of all types of cryoglobulins evaluated by us over the period 1960–1978. A characteristic clinical syndrome, consisting of recurrent palpable purpura (100 percent), polyarthralgias (72.5 percent) and renal disease (55 percent), was seen. Biopsy specimens of skin lesions showed cutaneous vasculitis, and half had immune reactants in vessel walls. Seventy percent of patients had evidence of hepatic dysfunction, often subclinical, and more than 60 percent of those tested had serologic evidence of prior infection with hepatitis B virus. Hepatic lesions ranged from minimal triaditis to chronic active hepatitis and/or cirrhosis. All 22 patients in whom clinical renal disease developed had significant proteinuria; 63.6 percent had diastolic hypertension, 77.3 percent edema, 45.5 percent renal failure and 22.7 percent were nephrotic. Glomerular disease associated with deposition of immunoglobulin G, immunoglobulin M and complement, often with coexistent renal arteritis, was confirmed pathlologically in 15 cases.All cryoglobulins had rheumatoid factor activity and consisted of IgM and polyclonal IgG; five also contained IgA. Thirteen had a monoclonal IgM kappa component. Serum protein electrophoresis was unremarkable or showed diffuse hyperglobulinemia. Striking depression of early complement components was noted but did not correlate well with the cryoprotein concentration, renal involvement or clinical course.Follow-up for periods up to 21 years from onset of symptoms revealed that renal involvement has a deleterious effect on prognosis. Postmortem examinations of nine patients demonstrated widespread vasculitis in addition to renal involvement. Preterminal infection was found in eight.</div>
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<affiliation>From the Departments of Medicine and Pathology, and the Irvington House Institute New York University School of Medicine, New York, New York.</affiliation>
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<abstract xml:lang="en"><p>The clinical course of 40 patients with significant quantities of mixed cryoglobulins, but without lymphoproliferative, collagen-vascular or chronic infectious diseases, is presented. These cases comprise 51.3 percent of all mixed and 31.7 percent of all types of cryoglobulins evaluated by us over the period 1960–1978. A characteristic clinical syndrome, consisting of recurrent palpable purpura (100 percent), polyarthralgias (72.5 percent) and renal disease (55 percent), was seen. Biopsy specimens of skin lesions showed cutaneous vasculitis, and half had immune reactants in vessel walls. Seventy percent of patients had evidence of hepatic dysfunction, often subclinical, and more than 60 percent of those tested had serologic evidence of prior infection with hepatitis B virus. Hepatic lesions ranged from minimal triaditis to chronic active hepatitis and/or cirrhosis. All 22 patients in whom clinical renal disease developed had significant proteinuria; 63.6 percent had diastolic hypertension, 77.3 percent edema, 45.5 percent renal failure and 22.7 percent were nephrotic. Glomerular disease associated with deposition of immunoglobulin G, immunoglobulin M and complement, often with coexistent renal arteritis, was confirmed pathlologically in 15 cases.All cryoglobulins had rheumatoid factor activity and consisted of IgM and polyclonal IgG; five also contained IgA. Thirteen had a monoclonal IgM kappa component. Serum protein electrophoresis was unremarkable or showed diffuse hyperglobulinemia. Striking depression of early complement components was noted but did not correlate well with the cryoprotein concentration, renal involvement or clinical course.Follow-up for periods up to 21 years from onset of symptoms revealed that renal involvement has a deleterious effect on prognosis. Postmortem examinations of nine patients demonstrated widespread vasculitis in addition to renal involvement. Preterminal infection was found in eight.</p>
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<head><ce:article-footnote><ce:label>☆</ce:label>
<ce:note-para>This work was supported by the Arthritis Foundation, Inc., U.S. Public Health Service Grants AM 02594, 07176, 01431 and the Michael and Helen Schaffer fund.</ce:note-para>
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<ce:author><ce:given-name>Yoram</ce:given-name>
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<ce:author><ce:given-name>Edward C.</ce:given-name>
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<ce:affiliation><ce:textfn>New York, New York USA</ce:textfn>
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<ce:correspondence id="COR1"><ce:label>∗</ce:label>
<ce:text>Requests for reprints should be addressed to Dr. Peter D. Gorevic.</ce:text>
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<ce:footnote id="FN2"><ce:label>∗</ce:label>
<ce:note-para>Present address: Division of Allergy, Rheumatology and Clinical Immunology, Department of Medicine, State University of New York, Stony Brook, New York 11794.</ce:note-para>
</ce:footnote>
<ce:footnote id="FN1"><ce:label>1</ce:label>
<ce:note-para>From the Departments of Medicine and Pathology, and the Irvington House Institute New York University School of Medicine, New York, New York.</ce:note-para>
</ce:footnote>
</ce:author-group>
<ce:date-accepted day="14" month="2" year="1980"></ce:date-accepted>
<ce:abstract class="author"><ce:section-title>Abstract</ce:section-title>
<ce:abstract-sec><ce:simple-para view="all" id="simple-para.0010">The clinical course of 40 patients with significant quantities of mixed cryoglobulins, but without lymphoproliferative, collagen-vascular or chronic infectious diseases, is presented. These cases comprise 51.3 percent of all mixed and 31.7 percent of all types of cryoglobulins evaluated by us over the period 1960–1978. A characteristic clinical syndrome, consisting of recurrent palpable purpura (100 percent), polyarthralgias (72.5 percent) and renal disease (55 percent), was seen. Biopsy specimens of skin lesions showed cutaneous vasculitis, and half had immune reactants in vessel walls. Seventy percent of patients had evidence of hepatic dysfunction, often subclinical, and more than 60 percent of those tested had serologic evidence of prior infection with hepatitis B virus. Hepatic lesions ranged from minimal triaditis to chronic active hepatitis and/or cirrhosis. All 22 patients in whom clinical renal disease developed had significant proteinuria; 63.6 percent had diastolic hypertension, 77.3 percent edema, 45.5 percent renal failure and 22.7 percent were nephrotic. Glomerular disease associated with deposition of immunoglobulin G, immunoglobulin M and complement, often with coexistent renal arteritis, was confirmed pathlologically in 15 cases.</ce:simple-para>
<ce:simple-para view="all" id="simple-para.0015">All cryoglobulins had rheumatoid factor activity and consisted of IgM and polyclonal IgG; five also contained IgA. Thirteen had a monoclonal IgM kappa component. Serum protein electrophoresis was unremarkable or showed diffuse hyperglobulinemia. Striking depression of early complement components was noted but did not correlate well with the cryoprotein concentration, renal involvement or clinical course.</ce:simple-para>
<ce:simple-para view="all" id="simple-para.0020">Follow-up for periods up to 21 years from onset of symptoms revealed that renal involvement has a deleterious effect on prognosis. Postmortem examinations of nine patients demonstrated widespread vasculitis in addition to renal involvement. Preterminal infection was found in eight.</ce:simple-para>
</ce:abstract-sec>
</ce:abstract>
</head>
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<mods version="3.6"><titleInfo lang="en"><title>Mixed cryoglobulinemia: Clinical aspects and long-term follow-up of 40 patients</title>
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<titleInfo type="alternative" lang="en" contentType="CDATA"><title>Mixed cryoglobulinemia: Clinical aspects and long-term follow-up of 40 patients</title>
</titleInfo>
<name type="personal"><namePart type="given">Peter D.</namePart>
<namePart type="family">Gorevic</namePart>
<namePart type="termsOfAddress">M.D.</namePart>
<affiliation>New York, New York USA</affiliation>
<description>Requests for reprints should be addressed to Dr. Peter D. Gorevic.</description>
<description>Present address: Division of Allergy, Rheumatology and Clinical Immunology, Department of Medicine, State University of New York, Stony Brook, New York 11794.</description>
<role><roleTerm type="text">author</roleTerm>
</role>
</name>
<name type="personal"><namePart type="given">Hannah Jane</namePart>
<namePart type="family">Kassab</namePart>
<namePart type="termsOfAddress">M.D.</namePart>
<affiliation>New York, New York USA</affiliation>
<description>From the Departments of Medicine and Pathology, and the Irvington House Institute New York University School of Medicine, New York, New York.</description>
<role><roleTerm type="text">author</roleTerm>
</role>
</name>
<name type="personal"><namePart type="given">Yoram</namePart>
<namePart type="family">Levo</namePart>
<namePart type="termsOfAddress">M.D.</namePart>
<affiliation>New York, New York USA</affiliation>
<description>From the Departments of Medicine and Pathology, and the Irvington House Institute New York University School of Medicine, New York, New York.</description>
<role><roleTerm type="text">author</roleTerm>
</role>
</name>
<name type="personal"><namePart type="given">Robert</namePart>
<namePart type="family">Kohn</namePart>
<namePart type="termsOfAddress">M.D.</namePart>
<affiliation>New York, New York USA</affiliation>
<description>From the Departments of Medicine and Pathology, and the Irvington House Institute New York University School of Medicine, New York, New York.</description>
<role><roleTerm type="text">author</roleTerm>
</role>
</name>
<name type="personal"><namePart type="given">Martin</namePart>
<namePart type="family">Meltzer</namePart>
<namePart type="termsOfAddress">M.D.</namePart>
<affiliation>New York, New York USA</affiliation>
<description>From the Departments of Medicine and Pathology, and the Irvington House Institute New York University School of Medicine, New York, New York.</description>
<role><roleTerm type="text">author</roleTerm>
</role>
</name>
<name type="personal"><namePart type="given">Philip</namePart>
<namePart type="family">Prose</namePart>
<namePart type="termsOfAddress">M.D.</namePart>
<affiliation>New York, New York USA</affiliation>
<description>From the Departments of Medicine and Pathology, and the Irvington House Institute New York University School of Medicine, New York, New York.</description>
<role><roleTerm type="text">author</roleTerm>
</role>
</name>
<name type="personal"><namePart type="given">Edward C.</namePart>
<namePart type="family">Franklin</namePart>
<namePart type="termsOfAddress">M.D.</namePart>
<affiliation>New York, New York USA</affiliation>
<description>From the Departments of Medicine and Pathology, and the Irvington House Institute New York University School of Medicine, New York, New York.</description>
<role><roleTerm type="text">author</roleTerm>
</role>
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<abstract lang="en">The clinical course of 40 patients with significant quantities of mixed cryoglobulins, but without lymphoproliferative, collagen-vascular or chronic infectious diseases, is presented. These cases comprise 51.3 percent of all mixed and 31.7 percent of all types of cryoglobulins evaluated by us over the period 1960–1978. A characteristic clinical syndrome, consisting of recurrent palpable purpura (100 percent), polyarthralgias (72.5 percent) and renal disease (55 percent), was seen. Biopsy specimens of skin lesions showed cutaneous vasculitis, and half had immune reactants in vessel walls. Seventy percent of patients had evidence of hepatic dysfunction, often subclinical, and more than 60 percent of those tested had serologic evidence of prior infection with hepatitis B virus. Hepatic lesions ranged from minimal triaditis to chronic active hepatitis and/or cirrhosis. All 22 patients in whom clinical renal disease developed had significant proteinuria; 63.6 percent had diastolic hypertension, 77.3 percent edema, 45.5 percent renal failure and 22.7 percent were nephrotic. Glomerular disease associated with deposition of immunoglobulin G, immunoglobulin M and complement, often with coexistent renal arteritis, was confirmed pathlologically in 15 cases.All cryoglobulins had rheumatoid factor activity and consisted of IgM and polyclonal IgG; five also contained IgA. Thirteen had a monoclonal IgM kappa component. Serum protein electrophoresis was unremarkable or showed diffuse hyperglobulinemia. Striking depression of early complement components was noted but did not correlate well with the cryoprotein concentration, renal involvement or clinical course.Follow-up for periods up to 21 years from onset of symptoms revealed that renal involvement has a deleterious effect on prognosis. Postmortem examinations of nine patients demonstrated widespread vasculitis in addition to renal involvement. Preterminal infection was found in eight.</abstract>
<note>This work was supported by the Arthritis Foundation, Inc., U.S. Public Health Service Grants AM 02594, 07176, 01431 and the Michael and Helen Schaffer fund.</note>
<note type="content">Section title: Review</note>
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