ANCA-associated vasculitis in scleroderma: A case series of fourteen patients

Kimberly P. Liang, Clement Michet

Research output: Contribution to journalReview article

6 Citations (Scopus)

Abstract

Antimyeloperoxidase (MPO), perinuclear antineutrophil cytoplasmic antibodies (pANCA), and/or clinically evident vasculitis in patients with scleroderma have been reported only rarely. The clinical significance and prognosis of ANCA-associated vasculitis in systemic sclerosis is uncertain. To report a case and identify the clinical characteristics of scleroderma patients with ANCA-associated vasculitis. Patients with both vasculitis and scleroderma occurring between 1976 to 2006 were identified using an electronic diagnostic index. These diagnoses were confirmed by retrospective review of complete medical records. Clinical features and outcomes recorded included age at vasculitis diagnosis, connective tissue disease (CTD) features, type of scleroderma (limited or diffuse); ANCA serology, vasculitic organ system manifestations; and death. Fourteen cases of scleroderma patients with ANCA-associated and/or small vessel vasculitis were identified. The majority (71%) were female, with mean age at vasculitis diagnosis 53 years. Seven patients (50%) had overlap CTD features, and the majority (79%) had limited variant of scleroderma. All of the 10 patients tested were MPO and pANCA positive. Seven patients (50%) had glomerulonephritis, 11 (79%) pulmonary involvement including 3 with pulmonary-renal syndrome, 6 skin purpura, and 5 mononeuritis multiplex and/or peripheral neuropathy. Six patients (43%) died during followup to 2008. The presence of pANCA-associated small vessel vasculitis is a rarely reported complication of scleroderma. It occurs most commonly in women with limited scleroderma and most commonly includes pulmonary and/or renal involvement, including severe organ-threatening manifestations and death. Further studies are needed to clarify the role and clinical impact of ANCA in scleroderma patients with and without vasculitis.

Original languageEnglish (US)
Pages (from-to)3-8
Number of pages6
JournalRheumatology Reports
Volume3
Issue number1
DOIs
StatePublished - Apr 1 2011

Fingerprint

Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis
Antineutrophil Cytoplasmic Antibodies
Vasculitis
Limited Scleroderma
Mononeuropathies
Diffuse Scleroderma
Lung
Connective Tissue Diseases
Purpura
Systemic Scleroderma
Peripheral Nervous System Diseases
Serology
Glomerulonephritis
Medical Records

Keywords

  • Antibodies
  • Antineutrophil cytoplasmic
  • Scleroderma
  • Vasculitis

ASJC Scopus subject areas

  • Immunology
  • Rheumatology

Cite this

ANCA-associated vasculitis in scleroderma : A case series of fourteen patients. / Liang, Kimberly P.; Michet, Clement.

In: Rheumatology Reports, Vol. 3, No. 1, 01.04.2011, p. 3-8.

Research output: Contribution to journalReview article

@article{d2a6a9478d1e4354a8283734c4de799b,
title = "ANCA-associated vasculitis in scleroderma: A case series of fourteen patients",
abstract = "Antimyeloperoxidase (MPO), perinuclear antineutrophil cytoplasmic antibodies (pANCA), and/or clinically evident vasculitis in patients with scleroderma have been reported only rarely. The clinical significance and prognosis of ANCA-associated vasculitis in systemic sclerosis is uncertain. To report a case and identify the clinical characteristics of scleroderma patients with ANCA-associated vasculitis. Patients with both vasculitis and scleroderma occurring between 1976 to 2006 were identified using an electronic diagnostic index. These diagnoses were confirmed by retrospective review of complete medical records. Clinical features and outcomes recorded included age at vasculitis diagnosis, connective tissue disease (CTD) features, type of scleroderma (limited or diffuse); ANCA serology, vasculitic organ system manifestations; and death. Fourteen cases of scleroderma patients with ANCA-associated and/or small vessel vasculitis were identified. The majority (71{\%}) were female, with mean age at vasculitis diagnosis 53 years. Seven patients (50{\%}) had overlap CTD features, and the majority (79{\%}) had limited variant of scleroderma. All of the 10 patients tested were MPO and pANCA positive. Seven patients (50{\%}) had glomerulonephritis, 11 (79{\%}) pulmonary involvement including 3 with pulmonary-renal syndrome, 6 skin purpura, and 5 mononeuritis multiplex and/or peripheral neuropathy. Six patients (43{\%}) died during followup to 2008. The presence of pANCA-associated small vessel vasculitis is a rarely reported complication of scleroderma. It occurs most commonly in women with limited scleroderma and most commonly includes pulmonary and/or renal involvement, including severe organ-threatening manifestations and death. Further studies are needed to clarify the role and clinical impact of ANCA in scleroderma patients with and without vasculitis.",
keywords = "Antibodies, Antineutrophil cytoplasmic, Scleroderma, Vasculitis",
author = "Liang, {Kimberly P.} and Clement Michet",
year = "2011",
month = "4",
day = "1",
doi = "10.4081/rr.2011.e2",
language = "English (US)",
volume = "3",
pages = "3--8",
journal = "Rheumatology Reports",
issn = "2036-7511",
publisher = "PagePress",
number = "1",

}

TY - JOUR

T1 - ANCA-associated vasculitis in scleroderma

T2 - A case series of fourteen patients

AU - Liang, Kimberly P.

AU - Michet, Clement

PY - 2011/4/1

Y1 - 2011/4/1

N2 - Antimyeloperoxidase (MPO), perinuclear antineutrophil cytoplasmic antibodies (pANCA), and/or clinically evident vasculitis in patients with scleroderma have been reported only rarely. The clinical significance and prognosis of ANCA-associated vasculitis in systemic sclerosis is uncertain. To report a case and identify the clinical characteristics of scleroderma patients with ANCA-associated vasculitis. Patients with both vasculitis and scleroderma occurring between 1976 to 2006 were identified using an electronic diagnostic index. These diagnoses were confirmed by retrospective review of complete medical records. Clinical features and outcomes recorded included age at vasculitis diagnosis, connective tissue disease (CTD) features, type of scleroderma (limited or diffuse); ANCA serology, vasculitic organ system manifestations; and death. Fourteen cases of scleroderma patients with ANCA-associated and/or small vessel vasculitis were identified. The majority (71%) were female, with mean age at vasculitis diagnosis 53 years. Seven patients (50%) had overlap CTD features, and the majority (79%) had limited variant of scleroderma. All of the 10 patients tested were MPO and pANCA positive. Seven patients (50%) had glomerulonephritis, 11 (79%) pulmonary involvement including 3 with pulmonary-renal syndrome, 6 skin purpura, and 5 mononeuritis multiplex and/or peripheral neuropathy. Six patients (43%) died during followup to 2008. The presence of pANCA-associated small vessel vasculitis is a rarely reported complication of scleroderma. It occurs most commonly in women with limited scleroderma and most commonly includes pulmonary and/or renal involvement, including severe organ-threatening manifestations and death. Further studies are needed to clarify the role and clinical impact of ANCA in scleroderma patients with and without vasculitis.

AB - Antimyeloperoxidase (MPO), perinuclear antineutrophil cytoplasmic antibodies (pANCA), and/or clinically evident vasculitis in patients with scleroderma have been reported only rarely. The clinical significance and prognosis of ANCA-associated vasculitis in systemic sclerosis is uncertain. To report a case and identify the clinical characteristics of scleroderma patients with ANCA-associated vasculitis. Patients with both vasculitis and scleroderma occurring between 1976 to 2006 were identified using an electronic diagnostic index. These diagnoses were confirmed by retrospective review of complete medical records. Clinical features and outcomes recorded included age at vasculitis diagnosis, connective tissue disease (CTD) features, type of scleroderma (limited or diffuse); ANCA serology, vasculitic organ system manifestations; and death. Fourteen cases of scleroderma patients with ANCA-associated and/or small vessel vasculitis were identified. The majority (71%) were female, with mean age at vasculitis diagnosis 53 years. Seven patients (50%) had overlap CTD features, and the majority (79%) had limited variant of scleroderma. All of the 10 patients tested were MPO and pANCA positive. Seven patients (50%) had glomerulonephritis, 11 (79%) pulmonary involvement including 3 with pulmonary-renal syndrome, 6 skin purpura, and 5 mononeuritis multiplex and/or peripheral neuropathy. Six patients (43%) died during followup to 2008. The presence of pANCA-associated small vessel vasculitis is a rarely reported complication of scleroderma. It occurs most commonly in women with limited scleroderma and most commonly includes pulmonary and/or renal involvement, including severe organ-threatening manifestations and death. Further studies are needed to clarify the role and clinical impact of ANCA in scleroderma patients with and without vasculitis.

KW - Antibodies

KW - Antineutrophil cytoplasmic

KW - Scleroderma

KW - Vasculitis

UR - http://www.scopus.com/inward/record.url?scp=79953155289&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=79953155289&partnerID=8YFLogxK

U2 - 10.4081/rr.2011.e2

DO - 10.4081/rr.2011.e2

M3 - Review article

AN - SCOPUS:79953155289

VL - 3

SP - 3

EP - 8

JO - Rheumatology Reports

JF - Rheumatology Reports

SN - 2036-7511

IS - 1

ER -