ACR Meeting Abstracts

ACR Meeting Abstracts

Abstract Number: 875

Differences in Early Damage Patterns in Various Forms of Primary Systemic Vasculitis

Katarzyna Wawrzycka-Adamczyk1,2, Joanna Robson3, Alberto Floris1,4, Jan Sznajd2,5, Anthea Craven6, Richard A. Watts7, Peter A. Merkel8, Raashid Luqmani5 and DCVAS investigators, 1Nuffield Department of Orthopaedics, Rheumatology and Musculoskeletal Sciences, University of Oxford, Oxford, United Kingdom, 2Jagiellonian University Medical College, Kraków, Poland, 3Rheumatology, University of Oxford, Oxford, United Kingdom, 4Rheumatology Unit, University Clinic, Cagliari, Italy, Monserrato, Italy, 5Nuffield Department of Orthopaedics Rheumatology and Musculoskeletal Sciences, University of Oxford, Oxford, United Kingdom, 6Nuffield Department of Orthopaedics Rheumatology and Musculoskeletal Sciences, Botnar Research Centre, University of Oxford, Oxford, United Kingdom, 7Rheumatology Department Ipswich Hospital and University of East Anglia, Ipswich, United Kingdom, 8Division of Rheumatology, University of Pennsylvania, Philadelphia, PA

Meeting: 2015 ACR/ARHP Annual Meeting

Date of first publication: September 29, 2015

Keywords:

Session Information

Date: Sunday, November 8, 2015

Title: Vasculitis Poster I

Session Type: ACR Poster Session A

Session Time: 9:00AM-11:00AM

Background/Purpose: Immunosuppressive agents have changed the outcome of the systemic vasculitides from invariably fatal to chronic conditions associated with damage as a result of disease, its treatment or comorbidity. The Vasculitis Damage Index (VDI) is a validated and standardized tool to measure damage occurring since the onset of disease. 

Methods: We compared patterns of damage amongst patients with 8 forms of primary vasculitis, using data obtained from the ACR/EULAR Diagnosis and Classification Criteria of Vasculitis study (DCVAS). VDI was evaluated at the follow up visit, at least 6 months from diagnosis. Total VDI scores, systems involvement and individual damage items were compared across different types of vasculitis. 

Results: The distribution of damage items was measured in 1371 adult patients with giant cell arteritis (GCA, n=453; median age 74 years [Inter Quartile Range 66-79]; Male:Female ratio 153:150), granulomatosis with polyangiitis (GPA; n=395; age 56 [43-65]; M:F 199:196), microscopic polyangiitis (MPA; n=158; age 65 [57-74]; M:F 70:88), eosinophilic granulomatosis with polyangiitis (EGPA; n=106; age 58 [44-65]; M:F 58:48), Takayasu‘s arteritis (TAK; n=93; age 33 [27-44]; M:F 21:72), IgA vasculitis (IgAV; n=86; age 46 [29-69]; M:F 49:37), Behçet‘s disease (BD; n=42; age 33 [28-39]; M:F 22:20), and polyarteritis nodosa (PAN; n=39; age 52 [34-61]; M:F 22:17). The median VDI score for all patients was 1.5 [0-11], measured at a mean of. 241±143 days after diagnosis. Levels of damage in different forms of vasculitis were defined as: low (IgAV and GCA, median VDI of 0; range 0-7), intermediate (PAN, GPA and BD, median VDI of 1; range 0-8) or high (TAK and EGPA, median VDI of 2; range 0-11). The most frequent VDI systems involved differed for each of the vasculitides included as follows: GCA: eyes (23%, p<0.001) and musculoskeletal (10%, p=0.82); GPA: ear, nose and throat (46%, p<0.001) and kidneys (22%, p<0.001); MPA: kidneys (53%, p<0.001) and lungs (25%, p<0.001); EGPA: lungs (58%, P<0.001) and neurological (57%, P<0.001); TAK: peripheral vessels (73%, p<0.001) and cardiovascular (27%, p<0.001); IgAV: kidneys (17%, p=0.46) and cardiovascular (6%, p=0.21); BD: skin/mucosa (56%, p<0.001) and eyes (21%, p=0.06); PAN: neurological (36%, p<0.001) and gut (20%, p<0.001). The most frequent individual items recorded in VDI per diagnosis are shown in Table 1.

Conclusion: Damage is detected early across the spectrum of systemic vascultides evaluated in this study, suggesting that patients suffer long term consequences of having a diagnosis of vasculitis.  The amount and pattern of damage differs between individual diseases. EGPA and TAK are associated with the highest levels of damage; IgAV and GCA have the lowest levels. These patterns could serve as a basis for evaluating the impact of new or existing therapies on outcomes in vasculitis.

Table 1 Most frequent items of damage recorded, six months after diagnosis of vasculitis

 

The top 3 most frequent VDI items recorded for each disease

Item

Frequency

P-value compared to all other forms of vasculitis

Giant cell arteritis

n=453

1. Visual impairment/diplopia

11%

p<0.001

2. Blindness in one eye

8%

p<0.001

3. Diabetes

7%

p=0.028

Granulomatosis with polyangiitis  

n=395

1. Nasal blockage/discharge/crusting

29%

p<0.001

2. Hearing loss

22%

p<0.001

3. eGFR<50%

15%

p<0.001

Microscopic polyangiitis  

n=158

1. eGFR<50%

38%

p<0.001

2. Proteinuria>0.5grams/24 hours

28%

p<0.001

3. Peripheral neuropathy

16%

p=0.007

Eosinophilic granulomatosis with polyangiitis  

n=106

1. Peripheral neuropathy

56%

p<0.001

2. Chronic asthma

44%

p<0.001

3. Chronic breathlessness

20%

p<0.001

Takayasu’s arteritis

n=93

1. Claudication>3months

47%

p<0.001

2. Major vessel stenosis

45%

p<0.001

3. Absent pulses in one limb

44%

p<0.001

IgA vasculitis  

n=86

1. Proteinuria>0.5grams/24 hours

16%

p=0.004

2. Cutaneus ulcers

5%

p<0.001

3. Osteoporosis

3%

p=0.46

Behçet’s disease

n=42

1. Mouth ulcers

52%

p<0.001

2. Visual impairment/diplopia

17%

p=0.002

3. Cataract

9%

p=0.048

Polyarteritis nodosa

n=39

1. Peripheral neuropathy

33%

p<0.001

2. Gut infarction

15%

p<0.001

3. Cutaneus ulcers

8%

p<0.001

Disclosure: K. Wawrzycka-Adamczyk, None; J. Robson, None; A. Floris, None; J. Sznajd, None; A. Craven, None; R. A. Watts, None; P. A. Merkel, None; R. Luqmani, GSK, 5,Chemocentryx, 5,Roche Pharmaceuticals, 5.

To cite this abstract in AMA style:

Wawrzycka-Adamczyk K, Robson J, Floris A, Sznajd J, Craven A, Watts RA, Merkel PA, Luqmani R. Differences in Early Damage Patterns in Various Forms of Primary Systemic Vasculitis [abstract]. Arthritis Rheumatol. 2015; 67 (suppl 10). https://acrabstracts.org/abstract/differences-in-early-damage-patterns-in-various-forms-of-primary-systemic-vasculitis/. Accessed .

« Back to 2015 ACR/ARHP Annual Meeting

ACR Meeting Abstracts - https://acrabstracts.org/abstract/differences-in-early-damage-patterns-in-various-forms-of-primary-systemic-vasculitis/