The journey of a patient
with small vessel vasculitis
Meet Mary she lives with one of the small vessel vasculitis conditions. Early diagnosis and appropriate timely access to treatment is essential to avoid irreversible organ damage. There are several different types of small vessel vasculitis, those which have anti-neutrophil cytoplasm antibodies – ANCA associated vasculitis (Granulomatosis with Polyangiitis, (GPA), Eosinophillic granulomatosis with Polyangiitis, (EGPA), Microscopic Polyangiitis, (MPA)) and non ANCA associated vasculitis (Henoch Schönlein Pupura/ IgA and Cryoglobulinemic Vasculitis). The main organs affected are kidneys, lungs and sinuses, but as these small vessel vasculides are systemic, there can be involvement of any organ.
Symptoms description
ENT
Sinusitis
Nose bleeds
Crusting
Repeated infections
Loss of hearing
Hoarseness
Saddle Nose
ORAL HEALTH
Bleeding gums
Ulcers
CARDIOLOGY
Arrythmia
Spasm of blood vessels
Heart failure
Heart Oedema
GASTRO-
ENTEROLOGY
Diarrhoea
Nausea
Bleeding
Pain
Perforated bowel (rare)
NEPHROLOGY
Haematurea
Frequent kidney infections
Urine foaming
Increased Blood pressure
NEUROLOGY
Loss or alteration of sensation Difficulty with co ordination Difficulty moving around
OPHTHALMOLOGY
Bloodshot eyes
Sensitivity to light
Inflammation of eye and eye structures
Conjunctivitis Changes to vision
RESPIRATORY
Wheeze
Difficulty breathing Cough
Coughing up blood Asthma
Repeated chest infections
DERMATOLOGY
Rash
Ulcers
Necrosis
NUTRITION
Loss of appetite
Loss of weight
INFECTIONS
Swollen glands
Feeling unwell Fever
Recurrent infections
not responding to antibiotic treatment
RHEUMATOLOGY
Joint pain
Joint swelling
Fatigue
Muscle pain
REQUIRED PRACTITIONERS
General /Healthcare Practitioner
Nurses
A range of organ specialists linked to main presenting symptoms
Emergency ward/ A&E/ ICU
Medical assessment unit
Physiotherapy
Dentist
Optician
Alternative medicines
Symptoms Challenges
HEALTHCARE PROFESSIONALS AWARENESS & EDUCATION
These are rare conditions so there is poor awareness of Vasculitis within the healthcare system especially primary care.
Symptoms come and go and a lot of symptoms are dismissed – regarded as psychosomatic.
Vasculitis mimics other illnesses.
ACCESS TO EARLY, ACCURATE DIAGNOSIS
Symptoms come and go and a lot of symptoms are dismissed – regarded as psychosomatic.
Risk of permanent organ damage without timely intervention.
No definitive diagnostic tool.
COMMUNICATION AND COORDINATION
These are systemic conditions resulting in many HCPs being involved in care. These may not always be aware of each other’s intervention.
HEALTH IMPACT
A wide range of very diverse physical symptoms.
Wide range of variation between individuals.
Symptoms can come and go.
Appears in childhood, adolescence as well as adulthood (peak incidence is age 50-60 years)
PSYCHOLOGICAL & SOCIAL IMPACT
Impact on both patient and carer
Anxiety, Depression Isolation and loss of self-confidence as symptoms increase and interfere with independence.
Financial challenges.
Absences from work or from school.
Costly health interventions.
QUALITY OF LIFE
Uncertainty re what is happening and what the future holds
Reduced independence
Symptoms Needs
HEALTHCARE PROFESSIONALS AWARENESS & EDUCATION
Increase awareness of small vessel vasculitis amongst the healthcare professionals.
Embedding Vasculitis in HCP and Medical student education modules.
Increase awareness and encourage attendance of existing specialist Vasculitis courses.
COMMUNICATION AND COORDINATION
Improve onward referral to other disciplines and improve communication between the same.
Collaboration between healthcare professionals involved in care to achieve earlier diagnosis and access to treatment.
Encourage holistic view of the patient, not the individual symptoms.
Symptoms can come and go.
DIGITAL HEALTH
All involved healthcare professionals to have access to health care records to ensure continuity of care.
SUPPORT & ADVOCACY
Support patient and family at this difficult time as they await a diagnosis .
Refer to local agencies who may be able to help with support and financial challenges.
Diagnostic description
ENT
Biopsy
Granulomas
Audiogram
CARDIOLOGY
Echocardiogram
GASTROENTEROLOGY
Colonoscopy
HEMATOLOGY
Raised ESR
Increased levels of white blood cells Increased CRP levels
Low HB (anaemia)
Positive ANCA test
NEUROLOGY
Nerve conduction tests
Electromyography test
OPHTHALMOLOGY
OpHthalmology tests
RESPIRATORY
Bronchoscopy
Respiratory tests
Lung granulomas
NEPHROLOGY
Proteinuria in urine
Red or white cells
in the urine
Kidney biopsy
IMAGING TECHNICS
X-ray, MRI, PET,
CT scans, Ultrasound
REQUIRED PRACTITIONERS
General/ Healthcare practitioner
Specialist discipline clinics depending on organ involvement
Emergency ward/Accident and Emergency / Medical assessment units
In patient units including Intensive care unit
Outpatient clinics
Private clinics
Radiology
Phlebotomy clinics
.
Diagnostic Challenges
HEALTHCARE PROFESSIONALS AWARENESS & EDUCATION
Lack of awareness leads to delayed access to appropriate tests, results and Diagnosis.
Unfortunately, small vessel vasculitis can mimic some illnesses which leads to misdiagnosis.
HEALTH IMPACT
Vasculitis mimics other illnesses.
A wide range of very diverse physical symptoms.
Symptoms can come and go.
Appears in childhood, adolescence as well as adulthood (peak incidence is age 50-60 years).
ACCESS TO EARLY, ACCURATE DIAGNOSIS
Delayed diagnosis can lead to permanent organ damage.
There is no definitive diagnostic test and so we rely on combining results from various tests to get an accurate picture.
Delayed diagnosis can lead to chronic repeat infections which reactivate the disease causing more damage.
COMMUNICATION AND COORDINATION
Many specialists are involved and need to have clear communication (digital health systems) in place to ensure holistic picture of patient and efficient use of available resources.
PSYCHOLOGICAL AND SOCIAL IMPACT
Impact on both patient and carer
Anxiety, Depression Isolation and loss of self-confidence as symptoms increase and interfere with independence.
Financial challenges.
Absences from work or from school.
Costly health interventions.
QUALITY OF LIFE
Uncertainty re what is happening and what the future holds
Reduced independence
Diagnostic Needs
HEALTHCARE PROFESSIONALS AWARENESS & EDUCATION
Increase awareness of signs of small vessel vasculitis amongst the healthcare professionals.
Embedding Vasculitis in HCP and Medical student education modules.
Increase awareness and encourage attendance of existing specialist Vasculitis courses.
COMMUNICATION & COORDINATION
Collaboration between clinics.
Clear explanations of what is happening to patient and carer.
QUALITY OF LIFE
Access to support services as outlined above.
Reassurance that one can recover a good quality of life with treatment.
RESEARCH
National and International cooperation – establishment of networks.
Data Sharing and interoperability.
Further research in genomics and biomarkers to establish clear diagnosis tools.
Registries
Reevaluation of diagnosis in time given the progress in the field including genomics and biomarkers.
ACCESS TO EARLY, ACCURATE DIAGNOSIS
Access to tests and prompt results. Timely intervention.
Access to specialist Clinicians/specialist small vessel vasculitis clinic.
SUPPORT & ADVOCACY
Patient needs to feel able to advocate for themselves to ensure getting the right diagnosis.
Signpost to relevant local services to help with all practicalities of diagnosis, physical, psychological and social.- including advise re work, education, or planning for early retirement.
Signpost to any benefit system that may assist with any associated financial costs of diagnosis.
Psychological support via counselor, psychologist, and/or self-help groups.
Uncertainty re what is happening and what the future holds
Reduced independence
Treatment description
IMMUNOSUPPRESSION
Immunosuppression
by Steroids,
Chemotherapy and
Biological drugs
DMARDS Disease
modifyin ganti-
rheumatic drugs
ANTIBIOTICS
either prophylactic
or to treat infection
PAIN, FATIGUE AND
BREATHLESSNESS
MANAGEMENT
KIDNEY DIALYSIS
AND TRANSPLANT
PSYCHOLOGICAL
SUPPORT
SURGICAL
INTERVENTION
PHYSIOTHERAPY
OCCUPATIONAL
THERAPY
ALTERNATIVE
THERAPIES
SENSORY SUPPORT
APPLIANCES
TREATMENT OF
SIDE EFFECTS
AND
PROPHYLACTIC
MEASURES
to prevent escalation
of same:ospeoporosis,
gastriculcers,
VACCINATIONS
Flu, Pneumonia,
Covid-19
PODIATRY
REQUIRED PRACTITIONERS
General/ Healthcare practitioner
Specialist discipline clinics depending on organ involvement
In patient clinics
Outpatient clinics
Phlebotomy clinics
Family planning clinics
Private clinics
Specialised nurses
Community nurses
Hospital and community pharmacists
Physiotherapist
Occupational Therapist
In Podiatrist
Dentist
Dietitian
Social worker
Psychological support
Alternative therapies clinics
Treatment Challenges
HEALTHCARE PROFESSIONALS AWARENESS & EDUCATION
Lack of awareness leads to delays in treatment and possible organ damage.
ACCESS
Sometimes special permission/approval/funding is required to access novel treatments
May need travel to clinics to have treatments and these may be some distance from home.
COMMUNICATION AND COORDINATION
Because so many different disciplines are involved, we need to have clear communication between the all disciplines involved.
CHRONICITY
OF TREATMENT
Immunosuppression leads to increased risk of infection and so restrict quality of life (education, employment, social situations)
Use of chemotherapy has implications on fertility and can induce early menopause.
Many treatments have side effects which need further monitoring /treating.
Long term use of steroids leads to development of cushingoid appearance.
Long term use of treatments leads to development of co morbidities i.e., osteoporosis, diabetes, glaucoma, increased risk of developing cancer.
QUALITY OF LIFE
Determined by organ involvement, independence, and ability to resume previous roles.
PATIENT EMPOWERMENT
Patient and their families are adjusting to a new life with small vessel vasculitis, how it impacts them, how the treatment impacts them and the changes that are needed in their lives. This is a very isolating and vulnerable time.
PSYCHOLOGICAL AND SOCIAL IMPACT
Lack of psychological support leads to anxiety re life changes, loss of independence, confidence, and self-esteem, stress related flare ups, and poor social interaction.
Treatment Needs
HEALTHCARE PROFESSIONALS AWARENESS & EDUCATION
Increase awareness of treatment of small vessel vasculitis amongst the healthcare professionals.
Embedding Vasculitis in HCP and Medical student education modules.
Increase awareness and encourage attendance of existing specialist Vasculitis courses.
PATIENT
ENGAGEMENT
Need to have clear communication with the patient and their carer to plan, explain and execute the appropriate treatment plan for the individual.
Information leaflets are usually available on the specific conditions and drugs, but these need reinforced by one-to-one discussion.to explain importance of drug /treatment regime, expected side effects, and precautions to be aware of.
The care team should be aware of the implications of small vessel vasculitis and its treatment on the life of the individual and their family –to this end the treatment plan should be a collaboration between the individual (and their family in the case of a child) and their clinicains.
ACCESS
Clear procedures in place to enable appropriate and timely access to current treatments.
COMMUNICATION AND COORDINATION
Clear established communication between all disciplines involved (i.e. electronic healthcare records).
CHRONICITY OF TREATMENT
Clear guidance on how to avoid exposure to infections.
Appropriate prophylactic measures in place to avoid development of co morbidities.
Advise re fertility, and options available re egg/sperm storage.
Advise on managing the menopause for women exposed to chemotherapy.
Treatment /management of side effects.
SUPPORT
AND ADVOCACY
Involvement in support group and/or self-help groups to empower the individual and their family in managing their condition and the related issues.
QUALITY OF LIFE
Determined by organ involvement, independence, and ability to resume previous roles.
Patient and their families are adjusting to a new life with small vessel vasculitis, how it impacts them, how the treatment impacts them and the changes that are needed in their lives. This is a very isolating and vulnerable time.
RESEARCH
Clinical trial to test new drugs/interventions and regimes of treatment to improve quality of life and manage small vessel vasculitis.
National and International cooperation – establishment of networks
Data Sharing and interoperability.
Further research to establish tools to identify flares, how to avoid flares and new treatments. – genomics, biomarkers
Registries to determine incidence, disease progression and effective treatments.
Follow up & Ageing description
INDIVIDUAL AWARE
of symptoms of relapse
and what to do when
felling unwell
DEVELOPMENT
OF CO
MORBIDITIES
and address issues
SHARED CARE
between specialist
clinics and general
healthcare practitioner
REFERRAL TO
FURTHER CLINICS
AS INDICATED
REGULAR REVIEWS
by general healthcare
practitioner, the named
specialist consultant,
and their team at all
discipline. Clinics involved
in care
REQUIRED PRACTITIONERS
General Healthcare practitioner.
In patient clinics
Out patient clinics
Specialist nurses
Community nursing
Pharmacist
Emergency ward
Healthcare professionals involved in care.
Social worker
Transition clinics
Gerontologist
Follow up & Ageing Challenges
MEDICAL
FOLLOW UP
Regular monitoring, access to test results and ability to quickly report symptoms to medical team to avoid unnecessary worry.
SUPPORT AND ADVOCACY
Access financial help/benefits and help to adjust to new life within the family.
Housing/school/workplace adaptations.
Physical/financial and psychological support systems in place
HCP FOLLOW UP
Multi system illness so need access to many different disciplines.
Access to HCPs as needed.
Access to Transition clinic for children.
Access to gerontology services in old age.
PATIENT EDUCATION
Awareness of what to do if symptoms change.
Awareness of how to manage condition by medication, lifestyle, and self-help.
CHRONICITY OF TREATMENT
Immunosuppression leads to increased risk of infection and so restrict quality of life (education, employment, social situations).
Use of chemotherapy has implications on fertility and can induce early menopause.
Many treatments have side effects which need further monitoring /treating.
Long term use of steroids leads to development of cushingoid appearance.
Long term use of treatments leads to development of co morbidities i.e., osteoporosis, diabetes, glaucoma, increased risk of developing cancer.
Follow up & Ageing Needs
MEDICAL
FOLLOW UP
Care needs to be coordinated by one clinician – rheumatologist, nephrologist or immunologist, and the individual patient. .
MEDICAL AWARENESS AND EDUCATION
Need to be aware of the changes that accompany aging and how to address these.
Plan for smooth transition of care from childhood services to adult and from adult to gerontology.
SUPPORT AND ADVOCACY
Support individual, carer and families in adjusting.
HCP FOLLOW UP
Good communication to exist between all involved in patient care.
PATIENT EDUCATION
Individuals and their family need to have more information on medications, treatments and living with small vessel vasculitis long term.
DIGITAL HEALTH
IT tools like dedicated smartphone apps allowing for real time reporting about disease symptoms and treatment complications to medical team.
Virtual clinics when condition is stable to avoid having to travel to busy clinics.
RESEARCH
Need registries to gather information on effective management of small vessel vasculitis, effective treatments, disease progression and geographical incidence.
Clinical trials.
Exploration of genomics and biomarkers in small vessel vasculitis.