REUMA.PT: MOVING FORWARD TO VASCULITIS
Ponte C1,2, Luqmani RA3, Mendonça S4, Martins FM4, Fonseca JE1,2, Canhão H1,2
E-mail: [email protected]
1
Rheumatology Department, Hospital de Santa Maria - Centro Hospitalar de Lisboa Norte, Lisbon Academic Medical Centre, Portugal
2
Rheumatology Research Unit - Instituto de Medicina Molecular, Faculdade de Medicina da Universidade de Lisboa, Portugal
3 Nuffield
Department of Orthopaedics Rheumatology and Musculoskeletal Sciences, Botnar Research Centre, University of Oxford, Oxford, United Kingdom
4 Sociedade
Portuguesa de Reumatologia
Introduction
• The Rheumatic Diseases Portuguese Register, Reuma.pt, was created in June 20081. By December 2013 over 10.000 patients had been inserted and
approximately 60.000 visits had been registered by 54 different centres, including 2 from Brazil.
• Reuma.pt still continues to expand and at the present includes specific protocols for seven different groups of rheumatic diseases.
• In 2012 a vasculitis outpatient clinic was created in Hospital de Santa Maria, Lisbon Academic Medical Centre, and with it a need to improve the register of this
subtype of rheumatic patients2. At the Nuffield Orthopaedic Centre in Oxford, a British centre with a special interest on systemic vasculitis, there was also a lack
of electronic medical tools to specifically register patients with this condition.
• Being vasculitis a group of relatively uncommon and complex diseases, the use of a dedicated electronic clinical record will improve its monitoring and clinical
care, while simultaneously increasing the knowledge of this field of Rheumatology.
Our aim is to describe the structure and functioning of the recent created Reuma.pt/Vasculitis.
Methods
Through a collaboration between Sociedade Portuguesa de Reumatologia and the Nuffield Orthopaedic Centre (Prof. Luqmani), specific classification and
assessment tools for patients with vasculitis were included in Reuma.pt/Vasculitis.
Results
Classification Criteria
General clinical data
ACR 1990 classification criteria
Chapel Hill 2012 nomenclature3
•
•
•
•
•
•
Giant cell arteritis
Takayasu arteritis
polyarteritis nodosa
Kawasaki disease
Granulomatosis with polyangiitis (Wegener’s)
Eosinophilic granulomatosis with polyangiitis
(Churg Strauss)
• Henoch–Schönlein purpura
• Large vessel vasculitis
• Medium vessel vasculitis
• ANCA-associated small vessel vasculitis
• Imunne complex small vessel vasculitis
• Variable vessel vasculitis
Others
• Single organ vasculitis
• Lanham 1984 criteria for Eosinophilic
Granulomatosis with Polyangiitis (Churg Strauss)
• International Study Group diagnostic criteria 1990
for Behçet’s disease
• Vasculitis associated with systemic disease
• Vasculitis associated with probable etiology
Cardiovascular risk
Five Factor Score
Original Five Factor Score (1996)
Yes No
Creatinine >140 µmol/L (1,6 mg/dL)
Score
Survival rate at 5 years
Proteinuria >1 g/24 h
Specific cardiomyopathy
• Vasculitis
o Onset of symptoms
o Diagnosis date
o Rheumatologist
• Smoking
• Alcohol consumption
• Specific medications
(known to be associated with vasculitis)
• Other drugs
Immunology
• Applicable to newly diagnosed patients with
Wegener´s Granulomatosis or Microscopic
Polyangiitis 4
Yes No UNK Date
c-ANCA on immunofluorescence
p-ANCA on immunofluorescence
PR3 ANCA (ELISA)
PR3 ANCA positive
MPO ANCA (ELISA)
Specific gastro-intestinal involvement
Specific central nervous system involvement
eGFR (MDRD)>32.3 mls/min
Age > 72.4 years
Revised Five Factor Score (2011)
Other ANCA
Please specify:_____________
Anti GBM antibodies
Yes No
Age >65 years
Cryoglobulins Type I (monoclonal
immunoglobulin)
Cryoglobulins Type II (monoclonal
RF (IgM) and polyclonal IgG)
Absence of ear, nose and/or throat
manifestations (GPA and EGPA only)
Cryoglobulins Type III (polyclonal
RF (IgM) and polyclonal IgG)
Creatinine >150 µmol/L (1,7 mg/dL)
Specific cardiomyopathy
Specific gastro-intestinal involvement
Score
Survival rate at 5 years
• SF-36
• EQD5
• FACIT
Any cardiovascular item
present on BVAS
Estimated risk:
Disease Activity
• BVAS
• VDI
> 1 mucous membrane
item present on BVAS
Disease Evolution
Assessment of disease activity
Assessment of damage
Specific
for Vasculitis
• The final graphics and charts include:
• The rest of the items not mentioned here
are very similar to the rest of the
Reuma.pt protocols
Disease activity indicators
• English and Portuguese versions are
available
Assessment of health
related quality of life
General
• The official launch of Reuma.pt/ Vasculitis
is planned for May/June 2014
Discussion and Conclusion
• This Reuma.pt/Vasculitis protocol has the potential to serve as an efficient daily tool for patients’ follow-up and data collection. It was done in collaboration with
an international referral centre highlighting the exponential growth and global recognition of Reuma.pt.
• In the context of this project the translation of Reuma.pt into the English language has also been started, making it more available for future research projects
and international.
REFERENCES
1- Canhão H, et al. Reuma.pt - the rheumatic diseases portuguese register.Acta Reum Port 2011;36:45-56.
2- Furtado C, et al. P63-Vasculites: experiência de um ano de consulta.Acta Reum Port 2013;38:80.
3- Jennette JC, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitis.Arthritis Rheum2013;65:1-11.
4- Suppiah R, et al . A model to predict cardiovascular events in patients with newly diagnosed Wegener's granulomatosis and microscopic polyangiitis.Arthritis Care Res.2011;63:588-96.