Harshna Richard Qadir
Assistant Professor, ACON, Amity University, Haryana.
*Corresponding Author E-mail: harshna.richard@gmail.com
ABSTRACT:
Churg Strauss syndrome (CSS) also known as Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare condition characterized by asthma, high levels of eosinophils (a type of white blood cell that helps fight infection), and inflammation of small to medium sized blood vessels (vasculitis).1 The inflamed vessels can affect various organ systems including the lungs, gastrointestinal tract, skin, heart and nervous system. No known cause of disease is found but believe to be an auto immune disorder and genetic factors and allergies play vital role to trigger the disease. CSS has 3 stages, with its own clinical manifestations. It’s not necessary that all patients with CSS will go through all 3 stages and they also do not manifest in same order always. The first stage is an allergic stage presents with respiratory problems, second stage is eosinophilic stage characterized by intra organ infiltration of eosinophils and final stage is a vasculitic stage with swollen lypmph nodes, joint pain, necrotizing inflammation of small vessels. Morbidity rate is high and it also need long term monitoring.
KEYWORDS: CSS, EGPA, autoimmune, genetic factor.
INTRODUCTION:
Churg-Strauss Syndrome (CSS) was first described in 1951 by Churg and Strauss. it is named after Drs. Jacob Churg and Lotte Strauss who, in 1951, first published about the syndrome by using the term "allergic granulomatosis" to describe it. It is a rare disease characterized by disseminated necrotizing vasculitis with extravascular granulomas occurring exclusively among patients with asthma and tissue eosinophilia. Churg-Strauss syndrome is counted as an extremely rare disease because that not only causes inflammation of blood vessels but, it can damage small and medium-sized blood vessels. Sometime physicians also call it Churg-Strauss vasculitis, eosinophilic granulomatosis with polyangiitis (EGPA), and allergic angiitis. It most often affects nose, sinuses, lungs, heart, intestines, and nerves. The inflammation can also affect the kidneys, muscles, or joints.
DEFINITION:
Churg-Strauss syndrome (CSS) is an extremely rare disease that causes inflammation of blood vessels. It can damage small and medium-sized blood vessels throughout the body. This can also result in asthma and blood cell abnormalities.2 It is also known as Churg-Strauss vasculitis, eosinophilic granulomatosis with polyangiitis, and allergic angiitis.3
EPIDEMIOLOGY4:
Prevalence: 1-2/1000,00
Male: Female 2-3:1
Mean age of disease onset is 40years
CAUSES5:
· The exact cause is unknown.
· Autoimmune, genetic, and environmental factors play a role.
· It is associated with allergies and certain medications that act as triggers.
· It is more commonly seen in patients of severe asthma
RISK FACTORS:
Anyone can get Churg-Strauss. But things that make it more likely include:
· Age: On average, people are between 30 and 50 when they get diagnosed.
· Asthma or nasal problems: Most people who get it have chronic asthma, nasal allergies, or chronic sinusitis.
SIGNS AND SYMPTOMS OF CHURG-STRAUSS SYNDROME6:
It includes the following:
· Asthma.(It is a chronic inflammatory disease of the airways that causes airway hyper responsiveness, mucosal edema, and mucus production.)
· 97% of patients have this symptom.
· Due to allergic stage (Allergy: is the strongest predisposing factor of asthma. Chronic exposure to airway irritants or allergens also increases the risk for developing asthma. Common triggers for asthma symptoms and exacerbations in patients with asthma include airway irritants (e.g., air pollutants, cold, heat weather changes, strong odours or perfumes, smoke), exercise, stress or emotional upsets
May be severe.
§ Allergic rhinitis (runny nose)
§ Sinus infection ( sinusitis )
§ Feeling unwell (malaise)
§ Fatigue.
§ Flu -like symptoms.
§ Weight loss.
§ Fever
Most common Signs and Symptoms of CSS
· The onset of allergies or the worseningof pre-existing allergies
§ Acute asthma
§ Sinus pain
§ Nasal polyps
§ Shortness of breath
§ Chest pain
§ Coughing up blood
§ Fever
§ Weight loss
§ Fatigue
§ Night sweats
§ Severe nerve pain in the arms and legs
§ Skin nodules
§ Diarrhea
§ Rash or skin sores
§ Seizures
§ Confusion
§ Joint pain
§ Irregular heartbeat
STAGES OF CSS7
CSS has 3 stages, with its own clinical manifestations. It’s not necessary that all patients with CSS will go through all 3 stages and they also do not manifest in same order always.
· Allergic stage
· Eosinophilic stage
· Vasculitic stage
PATHOPHYSIOLOGY WITH CLINICAL MANIFESTATION ACCORDING TO STAGES
Figure 1: Pathophysiology of Churg–Strauss syndrome (eosinophilic granulomatosis with polyangiitis).
DIAGNOSTIC EVALUATION8:
· H/O Asthma
· Eosinophilia greater than 10% on differential white blood cell count
· Mononeuropathy or polyneuropathy
· Paranasal sinus abnormality
· Non fixed pulmonary infiltrates on X-ray
· Biopsy containing a blood vessel with extravascular eosinophils
TREATMENT9
· Churg Strauss syndrome has no cure.
· Symptoms are highly varied,
· Specific medication to relieve the symptom of varied patient.
MEDICAL MANAGEMENT:
· Corticosteroids: To reduce inflammation. Prednisolone
· Immunosuppressant: Cyclophosphamide, azathioprine (Azasan, Imuran) or methotrexate (Trexall).
· Biological medications – Drugs like Rituxan/ rituximab helps to improve symptoms by alerting immune system and decrease the number of eosinophils
· Immune globulin – generally given to patient who haven’t responded to other treatments. Immune globulin is expensive and doesn’t work for everyone.
Churg-Strauss syndrome can affect many organs, including your lungs, skin, gastrointestinal system, kidneys, muscles, joints and heart. Without treatment, the disease can be fatal.
Complications, which depend on the organs involved, can include:
· Peripheral nerve damage. Churg-Strauss syndrome can damage the nerves in your hands and feet, leading to numbness, burning and loss of function.
· Skin scarring. The inflammation can cause sores that can leave scars.
· Heart disease. Heart-related complications of Churg-Strauss syndrome include inflammation of the membrane surrounding your heart, inflammation of the muscular layer of your heart wall, heart attack and heart failure.
· Kidney damage. If Churg-Strauss syndrome affects your kidneys, you can develop glomerulonephritis. This disease hampers your kidneys' filtering ability, leading to a buildup of waste products in your bloodstream.
NURSING MANAGEMENT11
Nurses are the vital member of health care team. And play a major role in caring and educating patients with EGPA because of its rarity, lifetime medication and life-threatening complication.
§ To promote patients health, self-awareness towards disease and follow up care and daily lifestyle modification, to increase in patient safety measures teaching is needed.
§ Clinical Nurse takes responsibility in treating a patient with EGPA i.e 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 need to be aware of the side effects of long-term glucocorticoid use and assess for these complications of patients with EGPA.
ACTUAL AND POSSIBLE NURSING DIAGNOSIS:
1. Ineffective airway clearance related to bronchospasm, retention of secretion and increased mucus.
2. Ineffective breathing pattern related to spasm of the airway and respiratory muscle fatigue.
3. Impaired gas exchange related to bronchospasm and damage to the alveoli.
4. Acute pain (joints and fingers) related to inflammation of the blood vessels secondary to vasculitis.
5. Hyperthermia related to infection.
6. Activity intolerance related to imbalance between oxygen supply and demand.
7. Imbalanced nutrition less than body requirement related to shortness of breath and activity intolerance.
8. Anxiety related to patient's perception of disease condition, crisis situation and threat to life.
9. Deficient knowledge related to new condition, procedure and treatment.
GOALS/ EXPECTED/OUTCOME:
· Airway patency is maintained need to be evidenced by clear breath sounds, improved oxygen exchange, normal rate and depth of respirations and ability to effectively cough out secretions.
· Optimal breathing pattern is maintained need to be evidenced by relaxed breathing, normal RR or pattern and absence of dyspnea.
· The patient maintains optimal gas exchange need to be evidenced by normal saturation levels, blood gases within normal range, clear lung fields and remains free of signs of respiratory distress.
· Pain is minimized need to be evidenced by reduction in the pain score, absence of facial grimacing.
· Optimal body temperature is maintained need to be evidenced by body temperature 37°C–37.5°C.
· He is able to perform basic activities without exertion dyspnea or fatigue or weakness.
· He maintains adequate nutritional status need to be evidenced by no further decline in strength and activity tolerance.
· He is able to remain calm, describe his own anxiety and coping pattern.
· He is able to understand the disease condition, treatment, medication, complications and importance of follow-up.
PROGNOSIS12:
· The prognosis is generally positive and has improved a great deal over the last 30 years. CSS can be treated successfully with combination of drugs.
· Aggressive treatment and close monitoring is needed. It means remission is possible for over 90 percent of patients.
· Early diagnosis and treatment is very important to achieve successful treatment.
· Some patient may have occasional relapse so prolonged and closed monitoring by patient and doctor is essential.
· Majority of treatment for CSS have side effects. So doctor tries to keep low doses of medication.
CONCLUSION:
CSS (EGPA) is a rare disease with no known cause. Autoimmune, genetic and environment are the risk factors. There is no cure for CSS at this time, but with early detection and treatment, patients can lead full, productive lives. They need to be on lifelong medication. Regular follow-up of medication and lifestyle modification can minimize the complication and improve the quality of life. Close monitoring is inevitable. Nurses play vital role in supporting and teaching the patients to adopt healthy lifestyle to remain free of pain and complications.
REFERENCES:
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8. Mayo Foundation for Medical Education and Research. Scottsdale; c1998-2019.Diagnosis,Treatmet, Available on : https://www.mayoclinic.org/diseases-conditions/churg-strauss-syndrome/diagnosis-treatment/drc-20353765
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10. Vasculitis Foundation. Eosinophilic Granulomatosis with Polyangiitis. Available on: https://www.vasculitisfoundation.org/education/forms/eosinophilic-granulomatosis-with-polyangiitis-churg-strauss-syndrome/
11. Blessing Reena Dason. Care of pateints with eosinophilic granulomatosis with polyangiitis. Nursing Management. Indian Journal of continuing Nursing education, October 09, 2019; Available on :https://www.ijcne.org/article.asp?issn=2230-7354;year=2019;volume=20;issue=1;spage=11;epage=17;aulast=Dason Churg-strauss syndrome prognosis, 2020. Available on:https://csssyndrome.com/churg-strauss-syndrome-prognosis/
Received on 23.06.2021 Modified on 17.03.2022
Accepted on 19.11.2022 © A&V Publications all right reserved
Int. J. Nur. Edu. and Research. 2023; 11(1):89-92.
DOI: 10.52711/2454-2660.2023.00020