Laboratory testing in the evaluation and diagnosis of vasculitis

Cuéllar, Marta Lucía; Espinoza, Luis R.

doi:10.1007/s11926-000-0042-6

Cited by 12 publications

(8 citation statements)

References 28 publications

(9 reference statements)

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“…Because the histopathological hallmark of ANCAassociated GN is pauci-immune necrotizing crescentic GN and AAV is generally not associated with hypocomplementemia (2,5), it was previously assumed that the complement system was not involved in the development of AAV. Recently, increasing evidences have suggested an important role of complement activation in the pathogenesis of AAV (6)(7)(8)(9)(10)(11)(12)(13).…”

Section: Introductionmentioning

confidence: 99%

Alternative Complement Pathway Activation Products in Urine and Kidneys of Patients with ANCA-Associated GN

Gou¹,

Yuan²,

Wang³

et al. 2013

Clinical Journal of the American Society of Nephrology

141

100

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SummaryBackground and objectives Previous study revealed that complement activation products of the alternative pathway could be detected in renal specimens of human ANCA-associated vasculitis. The current study aimed to investigate the clinical and pathologic significance of complement activation products in the urine and kidneys of patients with ANCA-associated vasculitis.Design, setting, participants, & measurements Renal biopsy specimens from 29 patients with ANCA-associated vasculitis diagnosed at Peking University First Hospital from January of 2008 to December of 2010 were randomly collected. Urine samples from 27 of 29 patients in active stage and 22 ANCA-associated vasculitis patients in complete remission who were independent of the above-mentioned 29 patients were collected. Urine samples from 28 patients with lupus nephritis and 25 healthy individuals were also collected. The renal deposition of Bb, C3d, and C5b-9 were detected by immunohistochemistry. The urinary levels of Bb, C3a, C5a, and soluble C5b-9 were determined by ELISA.Results The deposition, measured by the mean optical density of Bb, which is an alternative complement pathway marker, in glomeruli correlated with the proportion of total crescents (r=0.50, P=0.006), the extent of interstitial infiltrate (r=0.59, P=0.001), interstitial fibrosis (r=0.45, P=0.01), and tubular atrophy (r=0.55, P=0.002), whereas it correlated inversely with the proportion of normal glomeruli (r=20.49, P=0.008). The urinary levels of Bb, C3a, C5a, and soluble C5b-9 were all significantly higher in active compared with remission stage. The urinary levels of Bb in patients with active ANCA-associated vasculitis correlated with the serum creatinine (r=0.56, P=0.002) and correlated inversely with the proportion of normal glomeruli in renal specimens (r=20.49, P=0.009). ConclusionsThe present study provides additional evidence that complement activation through the alternative pathway occurred in the development of ANCA-associated vasculitis. The renal deposition of Bb and urinary Bb levels were associated with the severity of renal injury.

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Section: Introductionmentioning

confidence: 99%

Alternative Complement Pathway Activation Products in Urine and Kidneys of Patients with ANCA-Associated GN

Gou¹,

Yuan²,

Wang³

et al. 2013

Clinical Journal of the American Society of Nephrology

141

100

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“…The presence of anti-Sm antibodies was previously associated with a much higher incidence of both cutaneous and visceral vasculitis, especially when it co-existed with anti-dsDNA antibodies, as happened in this case. 6,10 Therefore, the patient had all the immunological manifestations that explain her fulminant and fatal clinical course. In this case it is very difficult to identify the factors that contributed to its fatal course.…”

Section: Discussionmentioning

confidence: 99%

Fulminant systemic vasculitis in systemic lupus erythematosus. Case report and review of the literature

Medina

González-Pérez

Vázquez-Juárez

et al. 2014

Lupus

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Vasculitis in systemic lupus erythematosus (SLE) has a broad spectrum of clinical manifestations from cutaneous to visceral involvement and its prognosis ranges from mild to life-threatening. We report the case of a previously healthy 17-year-old woman with eight months' history of arthralgias and myalgias. Subsequently, she developed facial and lower limbs edema, and hair loss. Two weeks before admission to a secondary level hospital, she developed fever up to 40°C followed by abdominal pain, rectal bleeding, hematemesis and blisters on both legs, reason for which she was hospitalized. With active bullous SLE with rapidly progressive glomerulonephritis suspected, she was treated with methylprednisolone pulses without response. After one week of treatment, she was transferred to a tertiary level hospital. On admission she presented acute arterial insufficiency of the lower extremities, respiratory failure with apnea, metabolic acidosis and shock; six hours later she died. Autopsy findings showed active diffuse lupus nephritis and diffuse systemic vasculitis that involved vessels from the skin, brain, myocardium, spleen, iliac and renal arteries. In addition, serositis of the small intestine and colon, acute and chronic pericarditis, pericardial effusion and myocarditis were found. Immunologic tests confirmed SLE diagnosis. In this case the fulminant course was the result of SLE high disease activity, visceral vasculitis of several organs and late diagnosis, referral and treatment. Early diagnosis, and opportune referral to the rheumatologist for intensive treatment can improve the outlook in these patients.

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“…22 The overall frequency of vasculitis in lupus varies between 11 and 20%. 1,6,[21][22][23][24][25][26][27][28][29][30] However, there is little data in the literature regarding cutaneous lesions of the digits in this disease. Bouaziz, et al reported 36% clinical vasculitis of the extremities in 50 consecutive patients with lupus, with histopathological confirmation in two cases.…”

Section: Discussionmentioning

confidence: 99%

Digital vasculitis in systemic lupus erythematosus: a minor manifestation of disease activity?

Gomes

Carvalho

Borba

et al. 2009

Lupus

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The objective of this study is to determine if digital vasculitis (DV), a clinical manifestation with a high systemic lupus erythematosus disease activity index (SLEDAI) score, is associated with lupus severity. DV and other clinical manifestations defined according to the SLEDAI were evaluated in 168 consecutive patients with systemic lupus erythematosus (SLE). Two groups were defined according to presence (DV+, n = 27) or absence of DV (DV-, n = 141) at the time of evaluation. The exclusion criterion was the presence of antiphospholipid syndrome (Sapporo's criteria). The two groups were comparable with regard to age (P = 0.09), gender (P = 1.00), white race (P = 0.81), and disease duration (P = 0.78). Compared to the DV- group, the DV+ group had a significantly higher frequency of mucocutaneous manifestations (66.7 vs. 39.0%, P = 0.01), haematological abnormalities (22.2 vs. 6.4%, P = 0.02) and constitutional symptoms (11.1 vs. 0.7%, P = 0.01). Renal and neurological involvements were similar in both groups (P = 0.57 and P = 1.00, respectively). The evaluation of each SLEDAI parameter confirmed that the DV+ group had higher frequencies of mild manifestations, such as new rash (P = 0.02), alopecia (P = 0.02), oral ulcers (P = 0.045), fever (P = 0.01) and leucopenia (P = 0.005). In contrast, both groups had similarly increased anti-dsDNA (P = 0.78) and decreased complement levels (P = 0.29). In conclusion, DV in patients with SLE identifies a subgroup of a mild disease. The high 'weighted' index attributed to this alteration in the SLEDAI score should therefore be revised.

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Laboratory testing in the evaluation and diagnosis of vasculitis

Cited by 12 publications

References 28 publications

Alternative Complement Pathway Activation Products in Urine and Kidneys of Patients with ANCA-Associated GN

Alternative Complement Pathway Activation Products in Urine and Kidneys of Patients with ANCA-Associated GN

Fulminant systemic vasculitis in systemic lupus erythematosus. Case report and review of the literature

Digital vasculitis in systemic lupus erythematosus: a minor manifestation of disease activity?

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