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Samter's Syndrome

Samter%27s Triad


Presentation

  • Her initial laboratory values are presented in Table 1.[ncbi.nlm.nih.gov]
  • The numbered bands specify the location of the thousands of genes that are present on each chromosome.[rarediseases.org]
  • These can also be present in cough or cold preparations, such as lozenges, so patients need to read the labels of these.[allergy.org.nz]
  • […] of all asthmatics and about 14% of adults with severe asthma. [5] AERD, which is slightly more prevalent in women, usually begins in young adulthood (twenties and thirties are the most common onset times, although children are afflicted with it and present[en.wikipedia.org]
Prolonged Bleeding
  • Platelet numbers are usually normal, but the platelets do not function properly causing easy bruising or prolonged bleeding. The disease can be categorized into classic and atypical (mild) forms.[rarediseases.org]
Inflammation
  • Approximately 10 per cent of the patients have urticaria and/or angioedema, alone or in combination with respiratory inflammation.[ncbi.nlm.nih.gov]
  • Usually, the first symptom is rhinitis (nasal inflammation), with sneezing, a runny nose, congestion, and often a reduced sense of smell. This is usually followed by the development of asthma, and then the growth of polyps inside the nose.[health24.com]
  • Most commonly the first symptom is rhinitis (inflammation or irritation of the nasal mucosa), which may manifest as sneezing, runny nose, or congestion.[en.wikipedia.org]
Pain
  • Thirty minutes after taking naproxen, the patient developed severe chest pain and came to the emergency department. She had previously experienced past episodes of chest pain after taking aspirin or nonsteroidal antiinflammatory drugs (NSAIDs).[ncbi.nlm.nih.gov]
  • Given that patients with AERD cannot use NSAIDs, they are sometimes prescribed a different type of anti-inflammatory for pain called a COX2 inhibitor. In one study of 172 patients with AERD, none reacted to a COX2 inhibitor.[allergy.org.nz]
  • In addition to the typical respiratory reactions, about 10% of patients with AERD manifest skin symptoms such as urticaria and/or gastrointestinal symptoms such as abdominal pain or vomiting during their reactions to aspirin. [2] In addition to aspirin[en.wikipedia.org]
Swelling
  • Hives and angioedema (swelling) are treated with antihistamines or corticosteroids. It is important to avoid aspirin and aspirin-containing medications.[health24.com]
Rhinitis
  • Usually, the first symptom is rhinitis (nasal inflammation), with sneezing, a runny nose, congestion, and often a reduced sense of smell. This is usually followed by the development of asthma, and then the growth of polyps inside the nose.[health24.com]
  • The symptoms The nasal symptoms are not just nasal polyps but can include rhinitis (inflammation of the nose) with sneezing, running of the nose, and congestion. Anosmia (loss of smell) is also typical.[allergy.org.nz]
  • (AIAR) [46] A person who has not yet experienced asthma or aspirin sensitivity might be diagnosed as having: Non-allergic rhinitis Non-allergic rhinitis with eosinophilia syndrome (NARES) History [ edit ] The syndrome is named for Max Samter. [47] Initial[en.wikipedia.org]
Sneezing
  • Usually, the first symptom is rhinitis (nasal inflammation), with sneezing, a runny nose, congestion, and often a reduced sense of smell. This is usually followed by the development of asthma, and then the growth of polyps inside the nose.[health24.com]
  • The symptoms The nasal symptoms are not just nasal polyps but can include rhinitis (inflammation of the nose) with sneezing, running of the nose, and congestion. Anosmia (loss of smell) is also typical.[allergy.org.nz]
  • Most commonly the first symptom is rhinitis (inflammation or irritation of the nasal mucosa), which may manifest as sneezing, runny nose, or congestion.[en.wikipedia.org]
Anosmia
  • This is absolutely essential if a patient has anosmia, sinusitis or nasal polyps, when the chances of reaction may be 40 per cent.[allergy.org.nz]
  • Anosmia (lack of smell) also is common, as inflammation within the nose and sinuses likely reaches the olfactory receptors. [8] The respiratory reactions to NSAIDs vary in severity, ranging from mild nasal congestion and eye watering to lower respiratory[en.wikipedia.org]
Cough
  • These can also be present in cough or cold preparations, such as lozenges, so patients need to read the labels of these.[allergy.org.nz]
  • […] common, as inflammation within the nose and sinuses likely reaches the olfactory receptors. [8] The respiratory reactions to NSAIDs vary in severity, ranging from mild nasal congestion and eye watering to lower respiratory symptoms including wheezing, coughing[en.wikipedia.org]
Nasal Congestion
  • Anosmia (lack of smell) also is common, as inflammation within the nose and sinuses likely reaches the olfactory receptors. [8] The respiratory reactions to NSAIDs vary in severity, ranging from mild nasal congestion and eye watering to lower respiratory[en.wikipedia.org]
Abdominal Pain
  • In addition to the typical respiratory reactions, about 10% of patients with AERD manifest skin symptoms such as urticaria and/or gastrointestinal symptoms such as abdominal pain or vomiting during their reactions to aspirin. [2] In addition to aspirin[en.wikipedia.org]
Vomiting
  • In addition to the typical respiratory reactions, about 10% of patients with AERD manifest skin symptoms such as urticaria and/or gastrointestinal symptoms such as abdominal pain or vomiting during their reactions to aspirin. [2] In addition to aspirin[en.wikipedia.org]
Urticaria
  • Approximately 10 per cent of the patients have urticaria and/or angioedema, alone or in combination with respiratory inflammation.[ncbi.nlm.nih.gov]
  • Analgesic Bee sting Exercise-induced anaphylaxis Anticoagulant Grass cutting Food allergy Antineoplastic Jellyfish sting Idiopathic anaphylaxis Bupropion Latex contact Mastocytosis Contrast media Poison ivy Serum sickness Corticosteroid Shellfish eating Urticaria[ncbi.nlm.nih.gov]
  • In addition to the typical respiratory reactions, about 10% of patients with AERD manifest skin symptoms such as urticaria and/or gastrointestinal symptoms such as abdominal pain or vomiting during their reactions to aspirin. [2] In addition to aspirin[en.wikipedia.org]
Angioedema
  • Approximately 10 per cent of the patients have urticaria and/or angioedema, alone or in combination with respiratory inflammation.[ncbi.nlm.nih.gov]
  • Hives and angioedema (swelling) are treated with antihistamines or corticosteroids. It is important to avoid aspirin and aspirin-containing medications.[health24.com]
  • Table 2 Causes of the Kounis-Zavras syndrome Condition Drug Environmental exposure Angioedema Antibiotic Ant sting Bronchial asthma Analgesic Bee sting Exercise-induced anaphylaxis Anticoagulant Grass cutting Food allergy Antineoplastic Jellyfish sting[ncbi.nlm.nih.gov]
Irritability
  • As with all allergic diseases, the cornerstone of treatment is environmental control with avoidance of respiratory irritants, aspirin, and aspirin-like medications.[ncbi.nlm.nih.gov]
  • Most commonly the first symptom is rhinitis (inflammation or irritation of the nasal mucosa), which may manifest as sneezing, runny nose, or congestion.[en.wikipedia.org]

Workup

Pericardial Effusion
  • During a repeat exercise stress echocardiogram, a small pericardial effusion was noted, and she was started on colchicine and naproxen.[ncbi.nlm.nih.gov]
Polyps
  • Polyps can be removed by surgery, although they often recur. Medications (corticosteroids) can also shrink the polyps. If they are not causing problems or discomfort, they may not need treatment.[health24.com]
  • AERD patients tend to have a large amount of polypoid tissue (ie polyps) and there is significant re-growth, with the average re-operation time for nasal polyps of just three years.[allergy.org.nz]
  • CASE REPORT A 25-year-old woman with previous asthma and nasal polyps presented to the emergency department with severe chest pain.[ncbi.nlm.nih.gov]
  • Desensitization to aspirin reduces the chance of nasal polyp recurrence and may slow the regrowth of nasal polyps. [26] Once desensitized to aspirin, most patients can safely take other NSAID medications again. [27] Even patients desensitized to aspirin[en.wikipedia.org]

Treatment

  • Treatment The separate parts of the triad are treated separately. Asthma treatment depends on severity: some patients require medication only during an attack, while others need to be on daily doses.[health24.com]
  • The results of current research are expected to lead to better understanding followed by further improvements in treatment for patients with Samter's syndrome.[ncbi.nlm.nih.gov]
  • The preferred treatment for many patients is desensitization to aspirin, undertaken at a clinic or hospital specializing in such treatment. [24] Patients who are desensitized then take a maintenance dose of aspirin daily to maintain their desensitization[en.wikipedia.org]

Etiology

  • Abstract The historic triad of bronchial asthma, nasal polyposis, and intolerance to aspirin and related chemicals, recently designated as Samter's syndrome, is an inflammatory condition of unknown etiology and pathogenesis.[ncbi.nlm.nih.gov]
  • Many etiologies of allergic angina pectoris have been reported (Table 2 ).[ncbi.nlm.nih.gov]

Pathophysiology

  • Mastocytosis Contrast media Poison ivy Serum sickness Corticosteroid Shellfish eating Urticaria Heparin Viper venom Intravenous anesthetic Wasp sting Lepirudin Nonsteroidal antiinflammatory drug Protamine Skin disinfectant Thrombolytic While the exact pathophysiology[ncbi.nlm.nih.gov]

Prevention

  • Before major procedures, a drug to prevent excessive bleeding, DDVAP, can be administered. Other than these options, treatment of CHS is symptomatic.[rarediseases.org]
  • Enteroimmunology: A Guide to the Prevention and Treatment of Chronic Inflammatory Disease. Carrabelle Florida: Psy Press. pp. 148–58. ISBN 978-1-938318-06-1. Amar YG, Frenkiel S, Sobol SE (February 2000).[en.wikipedia.org]

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