|Year : 2019 | Volume
| Issue : 1 | Page : 11-17
Care of patients with eosinophilic granulomatosis with polyangiitis
Blessing Reena Dason
Allergy and Immunology Unit, Hamad Medical Corporation, Doha, Qatar
|Date of Web Publication||09-Oct-2019|
Mrs. Blessing Reena Dason
Allergy and Immunology Unit, Hamad Medical Corporation, Post Box No. 3050, Doha
Source of Support: None, Conflict of Interest: None
Eosinophilic granulomatosis with polyangiitis formerly known as Churg–Strauss syndrome (CSS) is a multisystemic rare autoimmune disorder which results in restriction of blood flow to the vital organs, particularly the respiratory tract. CSS occurs in patients with a history of asthma or allergy. It has complex pathophysiology involving genetic factors, extrinsic agents and different cell types such as T-cells, oesinophils, B-cells, resident cells and anti-neutrophil cytoplasmic antibodies. Treatment mainly involves the administration of corticosteroids. Better knowledge about the disease condition and its treatment will assist nurses to educate the patients regarding the illness and prevention of further complication by adapting a healthy lifestyle such as regular exercise, Vitamin D supplement, diet control, smoking cessation and regular follow-up.
Keywords: Anti-neutrophil cytoplasmic antibodies, Churg–Strauss syndrome, corticosteroids
|How to cite this article:|
Dason BR. Care of patients with eosinophilic granulomatosis with polyangiitis. Indian J Cont Nsg Edn 2019;20:11-7
| Introduction|| |
Vasculitis is a disorder, in which there is inflammation of the blood vessels. It occurs when the body's immune system affects the blood vessels. Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare form of systemic vasculitis. It was formerly called as Churg–Strauss syndrome (CSS) and was first described by Churg and Strauss in 1951. It was later known as EGPA, allergic granulomatous angiitis or granulomatous small vessel vasculitis.
CSS or EGPA is a rare autoimmune disorder which causes inflammation of small and large blood vessels of patients associated with severe asthma, hypereosinophilia resulting in restricted blood flow and tissue damage of the vital organs. The international incidence of EGPA is approximately 2.5 cases/100,000 adults/year. In Asia, the annual incidence of EGPA varies between 0.37/million and 2.1/million population. The prevalence of EGPA globally is 10–15/million., The age at onset usually varies from 15 to 70 years.
| Aetiology And Risk Factors|| |
EGPA is thought to be an autoimmune disorder, and the exact cause is unknown. It may be due to heightened T-cell immunity elevated immunoglobulin (Ig) E and rheumatoid factor. The disease pathogenesis is not well known. It is probably the result of a complex interaction of genetic and environmental factors, with principal players being eosinophils, T- and B-lymphocytes that result in an inflammatory response.
The following are some of the risk factors, which may contribute to the illness.
- History of severe asthma
- History of nasal allergies
- Chronic sinusitis
- Medication uses (leukotriene receptor antagonists such as zafirlukast, montelukast, pranlucast, zileuton, use of recombinant anti-IgE monoclonal antibody omalizumab, use of low-to-high dose of oral steroids or inhaled steroids)
- Environmental exposure to inhaled allergens such as silica dust.
| Pathophysiology|| |
EGPA has a complex pathophysiology. The environmental factors, genetic factors and extrinsic factors along with T-cells, eosinophils, B-cells and resident cells participate in the inflammatory reaction and in the tissue injury. Overactivation of eosinophil's infiltrate tissues mainly blood vessels. The blood vessels become inflamed and restricted blood flow to vital organs and tissues. After prolonged periods of time, necrosis occurs from the lack of blood flow. Anti-neutrophil cytoplasmic antibodies (ANCAs) are autoantibodies produced by a person's immune system that attack proteins within the person's neutrophils. This can lead to neutrophilic degranulation and endothelial damage causing inflammation of small and large blood vessels [Figure 1].
|Figure 1:Pathophysiology of Churg–Strauss syndrome (eosinophilic granulomatosis with polyangiitis).|
Click here to view
| Clinical Manifestations|| |
EGPA has three stages, namely allergic stage, eosinophilic stage and vasculitic stage, each with its own signs and symptoms. Not all patients with EGPA go through all three stage of the disease and theStages always do not manifest in the same order [Figure 1].
| Diagnosing Criteria|| |
According to the American College of Rheumatology, there are six criteria for the diagnosis of EGPA. They are as follows:
- Asthma (wheezing and expiratory rhonchi)
- Eosinophilic count of ≥10% in peripheral blood
- Paranasal sinusitis
- Pulmonary infiltrates (may be transient)
- Histological confirmation of vasculitis with extravascular eosinophils
- Mononeuritis multiplex or polyneuropathy.
The presence of four out of six of these features has a high specificity and sensitivity for the diagnosis of EGPA/CSS. In 2012, International Chapel Hill Consensus Conference Nomenclature of Vasculitis has revised the following as the diagnosing criteria: eosinophil-rich and necrotising granulomatous inflammation often involving the respiratory tract and necrotising vasculitis predominantly affecting small-to-medium vessel and associated with asthma and eosinophilia.
| Investigations|| |
- Blood tests – It is done to detect ANCAs. This antibody will be positive for most of the patients with EGPA. They also have elevated eosinophils, creatinine, serum IgE and erythrocyte sedimentation rate
- Chest X-ray – Pulmonary opacity, transient pulmonary infiltrates and pleural effusion can be viewed in a chest X-ray showing vascular changes affecting lungs
- Pulmonary computed tomography (CT) – Peripheral areas of parenchymal consolidation with ground glass attenuation similar to chronic eosinophilic pneumonia can be seen in CT lungs
- Bronchiolar lavage – Lavage from bronchi in CT lungs will contain eosinophils
- Biopsy – Biopsy of the lung tissues shows necrotising vasculitis.
| Management|| |
Medications used to treat EGPA include:
- Corticosteroids – Prednisone is the common drug used for EGPA. Bone loss, hyperglycaemia, weight gain, cataracts and infections can occur as side effects of this drug
- Other immunosuppressive drugs – Cyclophosphamide, azathioprine (Azasan, Imuran) or methotrexate (Trexall) can be used to reduce body's immune reaction. If the patient has mild symptoms, then any one of the corticosteroid can be used
- Ig – For people who are not responding to other treatment, Ig is given as a monthly infusion. Igs are more expensive
- Biologic medications – Drugs such as rituximab (rituxan) can improve symptoms by altering the immune system and decrease the number of eosinophils.
| Complications|| |
EGPA can affect many organs, including the lungs, skin, gastrointestinal (GI) system, kidneys, muscles, joints and heart. Without treatment, the disease may be fatal. Complications depend on the organs involved and may include:
- Peripheral nerve damage – EGPA can damage the nerves in the hands and feet (peripheral neuropathy), leading to numbness, burning and loss of function
- Skin scarring – The inflammation may cause sores to develop that can leave scars on the skin
- Heart disease – Heart-related complications of EGPA include pericarditis, myocarditis, heart attack and heart failure
- Kidney damage – If EGPA affects the kidneys, it leads to glomerulonephritis. Kidney failure is uncommon.
| Prognosis|| |
The prognosis of this disease is determined by the following scoring system.
The presence of any of the five factors below contributes to poorer mortality and outcome.
- Proteinuria >1 g/24 h
- Serum creatinine >1.58 mg/dL (>140 μmol/L)
- GI tract involvement
- Central nervous system (CNS) involvement.
When none of the five factors are present (five-factor score [FFS] = 0), 5-year mortality is 11.9%. Mortality increases as the number of these five factors increases, with mortality increasing to 49.95% when at least two factors are present (FFS ≥2). Without treatment, the 5-year survival rate is about 25%. However, the patient outcomes have dramatically improved over a period of 20 years. Survival rates now exceed 90% at 1 year after diagnosis and 85% at 5 years. A diffuse organ involvement of EGPA, especially cardiovascular and rare involvement of the CNS and renal system suggests a poorer prognosis and can be fatal.
| Nursing Management|| |
Nurses play a major role in caring and educating patients with EGPA because of its rarity, lifetime medication and life-threatening complication. To promote their wellness, increase in patient safety measures and teaching is needed. The clinical responsibilities of a nurse in treating a patient with EGPA are to monitor their eosinophil levels, monitor their vital signs in case of fever or asthma, administer medications every day to help with management of their disease and establish the relationship by communication to help alleviate fear in patient and family. Patients with vasculitis will be on steroids for a prolonged period. Glucocorticoid-induced diabetes, osteoporosis, avascular necrosis, increased risk for infections, weight gain, cushingoid or moon faces, depression, progression of atherosclerosis, hypertension, glaucoma and cataracts are some of the potential side effects of corticosteroid use. Nurses should be aware of the side effects of long-term glucocorticoid use and assess for these complications of patients with EGPA.
| Case Report|| |
A case study of a patient with EGPA is discussed with the consent from him. Mr. X, 45-year-old male was admitted in the High Dependency Medical Unit with the complaints of shortness of breath for 2 days, fever with cough and rhinitis for 5 days, loss of weight and loss of appetite for 1 month and severe pain, numbness and tingling sensation in hands and feet for 1 week. He was an asthmatic for 5 years and was on steroid inhalers. On arrival, his temperature was 103°F, heart rate 102/min, respiratory rate (RR) 30/min, blood pressure 110/70 mmHg and SpO2 90%. Pain score was 9 on a 0–10 numerical pain rating scale. On examination, he appeared anxious and had paranasal sinusitis. Chest auscultation revealed bilateral wheeze and expiratory rhonchi. His chest X-ray showed pulmonary opacity with transient pulmonary infiltrates, and his pulmonary CT showed peripheral areas of parenchymal consolidation with ground glass attenuation. Electrocardiogram (ECG) showed normal sinus rhythm, and ECHO was normal with left ventricular ejection fraction of 64%. Blood investigation revealed the presence of ANCAs. The eosinophils count was 3000/ml with total IgE 1550 IU/ml, erythrocyte sedimentation rate of 70 mm at 1 h and total white blood cell (WBC) of 15,000/mm3. Arterial blood gas (ABG) revealed the presence of respiratory acidosis. Later, the lung biopsy was taken which revealed the necrotising vasculitis. He was started on oxygen 6 L through facemask. He was started on the following medication.
- Tablet prednisolone 20 mg bd
- Tablet paracetamol 1 g prn
- Tablet augmentin 625 mg Q8H
- Nebulised salbutamol 5 mg Q6H
- Nebulised ipratropium 0.5 mg Q6H
- Fluticasone inhaler 100 mcg bd
- Tablet dexamethasone 1 mg bd
- Tablet amitriptyline 10 mg hsod.
Ineffective airway clearance was related to bronchospasm, retention of secretion and increased mucus.
Airway patency is maintained as evidenced by clear breath sounds, improved oxygen exchange, normal rate and depth of respirations and ability to effectively cough out secretions.
- Assessed the RR, depth, rhythm and colour changes in the buccal mucosa, lips and nail beds. RR was 30/min and rhythmic. He had no significant colour changes in the buccal mucosa and lips, but had productive cough with white-coloured sputum
- Auscultated the lung for adventitious lung sounds and identified him to have expiratory wheeze and rhonchi
- Monitored the oxygen saturation level hourly, ABG values and chest X-ray results. SpO2 was 90%, and ABG showed respiratory acidosis, chest X-ray showed pulmonary opacity with transient pulmonary infiltrates and his pulmonary CT showed peripheral areas of parenchymal consolidation with ground glass attenuation
- Administered oxygen 6 L through facemask. Elevated the bed and positioned him in the Fowler's position
- Administered tablet dexamethasone 1 mg orally. Nebulisations with salbutamol and ipravent were administered every 6 h
- Encouraged him to use diaphragmatic breathing and practice coughing exercises
- Administered intravenous (IV) fluid as per order for adequate hydration
- Taught him to avoid respiratory irritants such as aerosols, cigarette smoke, fumes and extremes of temperature.
His airway patency was maintained as evidenced by the absence of adventitious lung sound such as rhonchi and wheeze, SpO299% and RR 18/min. He was able to cough and deep breathe purposefully q1–2 h during the day. Cough continued to be productive of moderately thin, white-coloured sputum.
Ineffective breathing pattern was related to spasm of the airway and respiratory muscle fatigue.
Optimal breathing pattern is maintained as evidenced by relaxed breathing, normal RR or pattern and absence of dyspnoea.
In addition to the above interventions for improving the patency of airway such as:
- Encouraged him to limit physical activity
- Taught him to do diaphragmatic breathing and pursed-lip breathing
- Assisted him in doing incentive spirometry.
Optimal breathing pattern was maintained as evidenced by relaxed breathing, normal RR or pattern RR 18/min and absence of dyspnoea evidenced by the absence of flaring of nostrils, chest retractions and use of accessory muscles.
Impaired gas exchange was related to bronchospasm and damage to the alveoli.
The patient maintains optimal gas exchange as evidenced by normal saturation levels, blood gases within normal range, clear lung fields and remains free of signs of respiratory distress.
- Assessed the RR, depth, rhythm, breathing pattern and use of accessory muscles. His RR was 30/min with rhythmic breathing and had no evidence of use of accessory muscles for breathing
- Monitored ABG which showed respiratory acidosis
- Monitored intake and output chart which showed a positive balance
- Assessed for cyanosis in nail beds and skin; colour of the tongue and oral mucous membranes identified that he had no significant colour changes
- Monitored oxygen saturation continuously, using pulse oximeter. Initially, his SpO2 on arrival was 90% and later with oxygen administration improved to 99%
- Administered humidified oxygen through facemask at 6 L/min
- Administered IV therapy as per order to ensure adequate hydration
- Encouraged slow deep breathing using an incentive spirometer
- Elevated the head of the bed and changed the patient's position every 2 h
- Encouraged the patient to limit physical activities and to rest. Assisted in the performance of activities of daily living
- Ensured adequate hydration.
He maintained optimal gas exchange as evidenced by normal ABG, absence of cyanosis, capillary refill >1 s, SpO298% and absence of adventitious lung sounds.
Acute pain (joints and fingers) was related to inflammation of the blood vessels secondary to vasculitis.
Pain is minimised as evidenced by reduction in the pain score, absence of facial grimacing.
- Assessed the quality, severity, location, onset, duration, precipitating and relieving factors of pain. He had dull nagging pain in all the joints and numbness and tingling sensation in the fingers. Pain score on the numerical scale was 9 initially
- Administered corticosteroid tablet prednisolone as ordered to minimise the inflammation
- Administered analgesic tablet paracetamol 1 g whenever he complained of pain as per order
- Administered tablet amitriptyline for neuropathic pain
- Explained the pain management protocols that are ordered, including therapies, medication administration, side effects and complications
- Reviewed the patient regularly after starting pain-relieving treatment and evaluated pain periodically
- Taught the use of nonpharmacological techniques (e.g., relaxation, guided imagery, music therapy, distraction and massage) along with other pain-relieving measures before, after and during painful activities.
He verbalised that his pain was minimised as evidenced by decrease in the pain score from 10/10 to 4/10 and absence of facial grimacing.
Hyperthermia was related to infection.
Optimal body temperature is maintained as evidenced by body temperature 37°C–37.5°C.
- Checked body temperature every 2 h. His temperature initially was 39.4°C
- Monitored the laboratory values and identified his WBC to be 15,000/mm3 initially
- Administered antipyretic tablet paracetamol 1 g prn as per physician's order
- Administered antibiotic tablet augmentin 625 mg tid as ordered
- Removed excess clothing and blankets and increased air circulation in the room
- Encouraged intake of oral fluids apart from IV fluids
- Provided air-conditioned room.
Optimal body temperature was maintained as evidenced by the temperature reduction from 39.4°C to <37.5°C with absence or chills.
Activity intolerance was related to imbalance between oxygen supply and demand.
He is able to perform basic activities without exertion dyspnoea or fatigue or weakness.
- Assessed his ability to perform activities and assisted him in performing self-care whenever needed
- Gradually increased activities and exercise; assisted him in doing passive to active and full range of motions
- Monitored his vital signs before and after activities to prevent falls and injuries from dizziness
- Observed the factors that were contributing to fatigue such as sweating
- Placed his knees and hips in an extended position to alleviate pain and provide relaxation
- Planed the nursing care with rest periods between activities
- Provided adequate periods for rest.
He was able to perform basic activities without dyspnoea or fatigue or weakness and maintains normal respiratory status during and after activities.
Imbalanced nutrition less than body requirement was related to shortness of breath and activity intolerance.
He maintains adequate nutritional status as evidenced by no further decline in strength and activity tolerance.
- Assessed the patient's knowledge on the importance and benefits of maintaining the normal nutritional body requirements
- Assessed for signs and symptoms of malnutrition. Physical examination revealed the loss of appetite and weight for the past 1 month
- Provided oral hygiene before meals
- Encouraged a rest period before meals to minimise fatigue
- Maintained a clean environment and relaxed, pleasant atmosphere
- Encouraged patient to eat frequent, small meals
- Allowed adequate time for meals
- Administered IV fluids as per order
- Limited fluid intake with food to reduce early satiety and subsequent decreased food intake
- Evaluated the patient's food preferences and diet recommendation
- Limited visitations during meal times.
There was no further decline in the strength and activity tolerance. He took adequate amount of food with appropriate calories.
Anxiety was related to patient's perception of disease condition, crisis situation and threat to life.
He is able to remain calm, describe his own anxiety and coping pattern.
- Assessed the current knowledge of situation to identify misconceptions, lack of information and other related issues
- Assessed the level of anxiety and identified that he remained anxious initially
- Noted the presence of insomnia, excessive sleeping and avoidance of interactions with others
- Identified the effectiveness of coping skills used by the patient currently
- Provided open and a trusting relationship. Spent time to listen and encouraged to voice concerns
- Used therapeutic communication skills of active listening, silence and acknowledgement
- Provided hope within parameters of the individual situation
- Acknowledged the awareness of his anxiety and encouraged him to express his feelings
- Provided calm, peaceful setting and privacy as appropriate. Promoted relaxation and ability to deal with the situation
- Provided time for him to meet with the doctor and share his concerns.
He was able to remain calm, express his anxiety and coping pattern.
Deficient knowledge was related to new condition, procedure and treatment.
He is able to understand the disease condition, treatment, medication, complications and importance of follow-up.
- Assessed his level of knowledge about specific disease processes
- Described the pathophysiology of the disease condition and how this relates to the anatomy and physiology, in a simple way
- Discussed the lifestyle changes that helps to control the disease or prevent complications in a understandable manner
- Importance of exercise – Explained about the side effects of corticosteroids such as weight gain and encouraged him to continue exercises such as walking and jogging which can help to maintain/reduce weight
- Importance of smoking cessation – As smoking can cause the symptoms of EGPA to worsen and increase the side effects of medication, stressed on the cessation of smoking
- Importance of healthy diet – As steroids can cause high blood sugar levels importance of maintaining a healthy diet to control the blood sugars was shared with the patient.
- Taught him about the importance of having regular cardiac monitoring and checkup (ECG and ECHO), because cardio toxicity is the most common life-threatening complication
- Discussed about the importance of follow-up and regular bone scan, undergoing eye examination, monitoring eosinophil level, blood sugar level and cholesterol monitoring
- Instructed him about the continuation of medication, especially the corticosteroids, Vitamin D and calcium supplements
- Taught him about the signs and symptoms that need to be reported at the appropriate time.
He verbalised that he understood about the disease condition and agreed to come for regular follow-up.
Mr. X was shifted to the ward on the 3rd day. His airway, breathing and circulation were normal. He was started on long-term corticosteroids. Pain medication and bronchodilators were continued. He was taught about the importance of regular follow-up and he agreed to comply.
| Conclusion|| |
EGPA is a rare autoimmune disorder. Patients with EGPA experience severe pain due to the disease process. They need to be on lifelong medications. Having regular follow-up, exercise and continuation of medications can minimise their complications and improve their span and quality of life. Nurses play a very important role in supporting and teaching the patients to adopt healthy lifestyle to remain free of pain and complications.
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Conflicts of interest
There are no conflicts of interest.
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