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Churg-Strauss Syndrome

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Churg-Strauss syndrome is a rare disorder that affects small- to medium-sized vessles. Patients often have an atopic background.


Presentation

In 1990, the American College of Rheumatology (ACR) proposed the following six criteria for the diagnosis of Churg-Strauss syndrome [5]:

The presence of 4 or more of these criteria is diagnosic of the disease [5].

Other common manifestations of Churg-Strauss syndrome are [1] [6]:


Churg-Strauss syndrome has 3 phases: allergic (rhinitis and asthma), eosinophilic infiltrative disease, and systemic vasculitis.

Fever
  • In every asthmatic patient with prolonged fever, eosinophilia and multisystemic involvment, CSS should be considered.[ncbi.nlm.nih.gov]
  • Thereafter, she was started on intravenous steroids, at which point the fever, pulmonary and cutaneous symptoms subsided.[ncbi.nlm.nih.gov]
  • Hay fever (allergic rhinitis). This affects the mucous membranes of your nose, causing a runny nose, sneezing and itching. Sinus pain and inflammation (sinusitis).[mayoclinic.org]
  • From Wikidata Jump to navigation Jump to search vasculitits that is systemic vasculitis realized as blood vessel inflammation and has_symptom asthma along with hay fever, rash and gastrointestinal bleeding.[wikidata.org]
  • We report the case of a 25-year old man who presented with a history of fever for two months, tingling, numbness, and paraesthesia of the upper limbs and left lower limb, along with diarrhoea for one month and an inability to walk for the past seven days[ncbi.nlm.nih.gov]
Weight Loss
  • Here we report the case of a 10-year-old girl who presented to us with vomiting, abdominal pain, and weight loss, paresthesias of lower extremities and breathlessness as well as a history of asthma, sinusitis and allergic rhinitis.[ncbi.nlm.nih.gov]
  • Cholestatic liver dysfunction has been reported. [ 8 ] Malaise, fatigue, weight loss, fever, myalgia and arthralgia.[patient.info]
  • Hypereosinophilia can result in: • Fever • Night sweats • Fatigue • Weight loss • Coughing • Abdominal pain • Sometimes gastrointestinal bleeding The third stage is the systemic vasculitis stage, marked by severe blood vessel inflammation in multiple[aaaai.org]
Weakness
  • Very few reports described myalgia or weakness as the chief complaint. Of these, only a few included muscle biopsy evaluation and none showed convincing evidence of primary myositis.[ncbi.nlm.nih.gov]
  • We report a case of Churg-Strauss syndrome with presenting symptoms of bilateral lower limb weakness and numbness only. The patient was admitted to an orthopaedic ward for management and a final diagnosis was reached following sural nerve biopsy.[ncbi.nlm.nih.gov]
  • A 72-year-old Japanese man, who had been previously diagnosed with asthma, presented with weakness of the left leg and purpura on the lower extremities.[ncbi.nlm.nih.gov]
  • RESULTS: The most common chief complaint was neuropathic limb pain and numbness (100%), followed by extremity weakness (82%). Nine (82%) patients had acute to subacute onset.[ncbi.nlm.nih.gov]
  • We report a 62-year-old man with mild fever, headache and acute visual loss in his right eye due to anterior ischaemic optic neuropathy (AION), followed a few days later by pain in the legs and left arm associated with numbness and weakness.[ncbi.nlm.nih.gov]
Fatigue
  • A 59-year-old man with general fatigue, numbness of limbs, and a 2-year history of asthma was admitted to the department of orthopedics.[ncbi.nlm.nih.gov]
  • Hypereosinophilia can result in: • Fever • Night sweats • Fatigue • Weight loss • Coughing • Abdominal pain • Sometimes gastrointestinal bleeding The third stage is the systemic vasculitis stage, marked by severe blood vessel inflammation in multiple[aaaai.org]
  • Signs and symptoms of the hypereosinophilia phase may include: Fever Loss of appetite and weight loss Night sweats Joint pain Asthma Fatigue Cough Abdominal pain and gastrointestinal bleeding Vasculitic stage The hallmark of this stage of Churg-Strauss[mayoclinic.org]
  • Churg-Strauss syndrome Pathology Type Autoimmune Cause(s) Unknown Symptoms allergies, asthma, sinusitis, fever, weight loss, fatigue, night sweats, cough, abdominal pain, rash, joint pain, peripheral neuropathy, Mortality Rate 25-50% after five years[house.wikia.com]
Fever of Unknown Origin
  • The patient had a fever of unknown origin, severe headache, progressing left ophthalmoplegia, and visual acuity disturbance.[ajnr.org]
  • Knockaert DC, Vanneste LJ, Bobbaers HJ (1993) Fever of unknown origin in elderly patients. J Am GeriatrSoc 41: 1187 Google Scholar 56.[link.springer.com]
Hemoptysis
  • A young male with complaints of cough, dyspnea and hemoptysis was admitted. He was using fluticasone propionate and salmeterol for two years for his asthma.[ncbi.nlm.nih.gov]
  • If the lungs are involved the following symptoms might be present: cough, shortness of breath, hemoptysis (coughing up blood stained sputum), rales (small clicking, bubbling or rattling sounds in the lung), rhonchi (chest sounds that sound like snoring[cssassociation.org]
  • Cough and hemoptysis, due to alveolar hemorrhage, may be present. Transient patchy pulmonary infiltrates are common. Neurologic: Neurologic manifestations are very common.[merckmanuals.com]
  • Hoffmann , Seltene Ursache einer Hämoptyse Rare cause of hemoptysis , Monatsschrift Kinderheilkunde , 10.1007/s00112-018-0446-x , (2018) . Karen Small, Andrew Stephenson and C.[doi.org]
Rales
  • If the lungs are involved the following symptoms might be present: cough, shortness of breath, hemoptysis (coughing up blood stained sputum), rales (small clicking, bubbling or rattling sounds in the lung), rhonchi (chest sounds that sound like snoring[cssassociation.org]
Abdominal Pain
  • A 66-year-old Japanese male presented with a history of recurrent abdominal pain. A diagnostic work-up revealed eosinophilia, eosinophilic gastritis, eosinophilic pneumonia, and SIgAD over 1 year of clinical observation.[ncbi.nlm.nih.gov]
  • The most common symptoms are abdominal pain, diarrhoea and occasionally gastrointestinal bleeding and perforation. We present a case of Churg-Strauss syndrome with small bowel lesions documented by video capsule endoscopy.[ncbi.nlm.nih.gov]
  • Here we report the case of a 10-year-old girl who presented to us with vomiting, abdominal pain, and weight loss, paresthesias of lower extremities and breathlessness as well as a history of asthma, sinusitis and allergic rhinitis.[ncbi.nlm.nih.gov]
  • Hypereosinophilia can result in: • Fever • Night sweats • Fatigue • Weight loss • Coughing • Abdominal pain • Sometimes gastrointestinal bleeding The third stage is the systemic vasculitis stage, marked by severe blood vessel inflammation in multiple[aaaai.org]
Loss of Appetite
  • Depending on what organs of the body are affected symptoms can include: Flu-like symptoms including a fever and general feeling of being weak and tired Loss of appetite Weight loss Muscle pain Causes and Risk Factors It is not yet know what causes this[cedars-sinai.edu]
  • Signs and symptoms of the hypereosinophilia phase may include: Fever Loss of appetite and weight loss Night sweats Joint pain Asthma Fatigue Cough Abdominal pain and gastrointestinal bleeding Vasculitic stage The hallmark of this stage of Churg-Strauss[mayoclinic.org]
  • […] of appetite, fever, and muscle pain (myalgia), Serious organ damage and potentially life-threatening complications.[symptoma.com]
Heart Failure
  • It may be difficult to determine myocardial disease activity status versus tissue damage in CSS with prolonged duration of heart failure symptoms.[ncbi.nlm.nih.gov]
  • We report a case of CSS masquerading as a non-ST elevation myocardial infarction with heart failure.[ncbi.nlm.nih.gov]
  • Four months later, the patient was hospitalized due to congestive heart failure with neuropathy of both upper extremities. A diagnosis of eosinophilic myocarditis was made based on the patient's laboratory results and the presence of mural thrombus.[ncbi.nlm.nih.gov]
  • However, sometimes the other reasons for acute coronary syndrome and heart failure are found. One of such reasons is hypereosinophilia which can be recognized if number of blood eosinophils exceeds 1500/mm3.[ncbi.nlm.nih.gov]
  • Here we report a case of CSS presenting with acute myocarditis and heart failure and review the literature on CSS with cardiac involvement.[ncbi.nlm.nih.gov]
Purpura
  • A 67-year-old woman with asthma visited our hospital with increasing dyspnea and new-onset paresthesia and purpura in her legs. Physical examination showed a wheeze, pretibial edema, and surrounding purpura.[ncbi.nlm.nih.gov]
  • She developed polyarthralgia, headache and purpura. A laboratory workout found hypereosinophilia (1150/µL), positive p-ANCA, microscopic haematuria and proteinuria at 2g/day.[ncbi.nlm.nih.gov]
  • We report a case of CSS with manifestations that included skin purpura and severe peripheral nerve degeneration in a 56-year-old woman with a recent history of asthma.[ncbi.nlm.nih.gov]
  • A 72-year-old Japanese man, who had been previously diagnosed with asthma, presented with weakness of the left leg and purpura on the lower extremities.[ncbi.nlm.nih.gov]
  • Cardiac involvement is common. [ 6 ] This includes heart failure, myocarditis and myocardial infarction. [ 7 ] Skin: purpura, skin nodules, leukocytoclastic angiitis with palpable purpura, livedo reticularis, urticaria, necrotic bullae and digital ischaemia[patient.info]
Palpable Purpura
  • She had conjunctival pallor, gangrene involving all fingertips of the left hand, palpable purpura on the anterior aspect of the left leg, and 3/5 grade motor weakness of the right wrist joint and left ankle joint on dorsiflexion.[mja.com.au]
  • Cardiac involvement is common. [ 6 ] This includes heart failure, myocarditis and myocardial infarction. [ 7 ] Skin: purpura, skin nodules, leukocytoclastic angiitis with palpable purpura, livedo reticularis, urticaria, necrotic bullae and digital ischaemia[patient.info]
  • Tlacuilo-Parra et al 7 similarly reported cutaneous manifestations, including palpable purpura, petechiae, nodules, maculopapules, and livedo reticularis, in approximately 51% of patients with CSS.[jamanetwork.com]
Polyneuropathy
  • Further tests verified pulmonary infiltrates, paranasal sinusitis, extravascular eosinophils on histopathologic sample of skin tissue, and polyneuropathy.[ncbi.nlm.nih.gov]
  • A 69-year-old male had an 8-year history of bronchial asthma and chronic sinusitis with hypereosinophilia (35 %), polyneuropathy, and a positive antineutrophil cytoplasmic antibody titer, so he was diagnosed with CSS.[ncbi.nlm.nih.gov]
  • A 58-year-old male presented with sensory motor polyneuropathy and rapidly progressive renal failure. Investigations revealed marked peripheral eosinophilia and elevated perinuclear antineutrophil cytoplasmic antibody titers.[ncbi.nlm.nih.gov]
  • Combining clinical and electrophysiological data, 10 (91%) patients demonstrated notable asymmetric involvement, whereas 1 patient presented with a length-dependent symmetrical axonal polyneuropathy.[ncbi.nlm.nih.gov]
  • For the traditional format classification, 6 criteria were selected: asthma, eosinophilia greater than 10% on differential white blood cell count, mononeuropathy (including multiplex) or polyneuropathy, non-fixed pulmonary infiltrates on roentgenography[ncbi.nlm.nih.gov]
Sensory Deficit of the Lower Extremity
  • The patient was admitted in our hospital with fever, malaise, sensory deficits in the lower extremities, diffuse musculoskeletal and thoracic pain.[ncbi.nlm.nih.gov]
Hematuria
  • Over a follow-up of 2 months, he has no hematuria/eosinophilia and serum creatinine of 2.3 mg/dL has decreased to 1.7 mg/dL.[ncbi.nlm.nih.gov]
  • […] numbness and tingling in your hands and feet (peripheral neuropathy) Severe abdominal pain Diarrhea, nausea and vomiting Shortness of breath (dyspnea) from asthma or congestive heart failure Cough Chest pain Irregular heartbeat Blood in your urine (hematuria[mayoclinic.org]
  • Urinalysis shows abnormal urine sediment, proteinuria, microscopic hematuria, and red blood cell casts with renal involvement. Imaging Pulmonary opacities can be found in 26 - 77% of cases. Pulmonary infiltrates may be transient.[symptoma.com]
Kidney Failure
  • Kidney failure is uncommon. Aug. 16, 2016[mayoclinic.org]
  • Kidney failure is uncommon. Diagnosis No specific test can confirm Churg-Strauss syndrome. And signs and symptoms are similar to those of other diseases, so it can be difficult to diagnose.[drugs.com]

Workup

Laboratory Studies

  • Complete blood count shows eosinophilia (usually at least 10% or 5000-9000 eosinophils/µL), and mild anemia.
  • Erythrocyte sedimentation rate (ESR) is elevated.
  • C-reactive protein (CRP) levels are also elevated.
  • Renal tests - Elevated serum blood urea nitrogen (BUN) and creatinine levels in cases of renal involvement .
  • Antineutrophil cytoplasmic antibodies (ANA) are present in approximately 40% of patients.
  • Serum IgE levels are elevated.
  • Rheumatoid factor is elevated at low titer levels.

Urinalysis shows abnormal urine sediment, proteinuria, microscopic hematuria, and red blood cell casts with renal involvement.

Imaging

Pulmonary opacities can be found in 26 - 77% of cases. Pulmonary infiltrates may be transient [2]. Computer Assisted Tomography (CT) findings include areas of consolidation similar to that of chronic pneumonia. Bronchial dilatation and wall thickening may also be seen [2].

Other tests may be indicated for specific organ-system involvement:

Biopsy

If local organ involvement exists, obtaining a biopsy of that organ is helpful in confirming the diagnosis. Biopsies of skin, lung,
kidney, nerve, or muscle may be indicated.

Albuminuria
  • In Churg and Strauss' original report, they comment that mild hematuria and albuminuria were commonly present ( 5 ). Three of their 11 patients had azotemia and one died from uremia.[jasn.asnjournals.org]

Treatment

Churg-Strauss syndrome is a systemic disorder that involves multiple organ systems. Therefore, the type of treatment required depends on the type of organ system involvement.

Glucocorticoids alone are usually adequate for the treatment of Churg-Strauss syndrome. Corticosteroids modify the immune response. Oral prednisone is usually sufficient to control the vasculitis.

Cytotoxic drugs are necessary in less than 20% of patients [8]. Cytotoxic agents inhibit cell growth and proliferation of eosinophils. They are reserved for cases that do not respond to corticosteroids. Cyclophosphamide (Cytoxan, Neosar) is the drug most often used.

Prognosis

With treatment, the 1-year survival rate is 90% and the 5-year survival rate is 62%.

Etiology

Churg and Strauss first described the syndrome in 1951. They found the disorder in 13 patients who had asthma as well as eosinophilia, inflammation, systemic vasculitis, and glomerulonephritis [3].

The cause of Churg-Strauss syndrome is unknown. However, the presence of hypergammaglobulinemia, increased levels of immunoglobulin E (IgE), rheumatoid factor, and anti-neutrophil cytoplasmic antibody, [4] in these patients suggested an allergic autoimmune disorder.

Epidemiology

The incidence of Churg-Strauss syndrome in the United States is 1 to 3 cases per 100,000 adults per year [2]. The international incidence of Churg-Strauss syndrome is approximately 2.5 cases per 100,000 adults per year [2].

Churg-Strauss syndrome is slightly more common in males than in females.

The age at onset varies from 15-70 years, with a mean age of approximately 38 years.

Sex distribution
Age distribution

Pathophysiology

Churg-Strauss syndrome is a systemic vasculitis in which the lung is the predominant, but not the only, site of inflammation. Churg-Strauss syndrome is a rare form of systemic vasculitis occurring only in patients with asthma [5].

The vascular inflammation in the disorder results from a prolonged life of eosinophils in these individuals. Although the exact mechanisms involved in Churg-Strauss syndrome have not been identified, it seems to be the secretion eosinophil-activating cytokines  [4] by T-lymphocytes that is the stimulating factor.

Differential diagnoses include Wegener's granulomatosis and other forms of systemic vasculitis [6]. Some recent studies show Churg-Strauss syndrome developing when oral steroid doses are reduced and a leukotriene receptor antagonist (montelukast, zafirlukast) is added [7].

Prevention

There are no guidelines for prevention of Churg-Strauss syndrome.

Summary

Churg-Strauss syndrome is a rare, systemic vasculitis that affects small- to medium-sized arteries and veins. It is a multi-system disorder that affects primarily the respiratory system, but can also involve the cardiovascular and renal systems.

Churg-Strauss syndrome is characterized by a history of asthma, elevated eosinophilia count, and necrotizing vasculitis with inflammation [1].

The long-term prognosis for Churg-Strauss syndrome is good. Most patients need low dose oral corticosteroids for persistent asthma. Some need continued anti-inflammatory medication for many years after clinical recovery from vasculitis [1] to prevent recurrence.

The principal cause of morbidity and mortality in Churg-Strauss syndrome is myocarditis and myocardial infarction, secondary to coronary arteritis [2].

Patient Information

What is Churg-Strauss syndrome?

Churg-Strauss syndrome is a rare disorder that may affect multiple organ systems, especially the lungs. It is a type of vasculitis (blood vessel inflammation) that occurs throughout the body.

It is an auto-immune disorder, meaning the body's white blood cells, which usually fight off infection, begin attacking healthy tissue.

Churg-Strauss syndrome occurs almost always in individuals who have a history of asthma.

What are the symptoms of Churg-Strauss syndrome?

Churg-Strauss Syndrome has many symptoms involving many different organ systems. A person with Churg Strauss syndrome may have some or all of the following symptoms [9]:

Besides the symptoms listed above, the person with Churg Strauss syndrome may also have:

  • Nodules or purpura (purplish blotches) on the skin,
  • Damage to the kidneys, heart, or other organs because of inflammation of their blood vessels,
  • Generalized flu-like symptoms, such as weakness, fatigue, loss of appetite, fever, and muscle pain (myalgia),
  • Serious organ damage and potentially life-threatening complications.

Who gets Churg-Strauss syndrome?

Most people with Churg-Strauss Syndrome have a history of asthma or other allergy. Usually, a person already has asthma when they develop Churg Strauss syndrome.

The systemic symptoms may follow an allergic reaction by six months or more.
Churg-Strauss Syndrome affects all age groups, but children more commonly. Males and females are equally affected.

How is Churg-Strauss syndrome treated?

Churg-Strauss Syndrome is an autoimmune disorder. This means that the body’s immune system mistakenly attacks itself.

Treatment is aimed at slowing the immune response. The most commonly used medication is prednisone, an immunosuppressant. It is given orally, except in severe cases when it is given intravenously. The medication usually works in a few days. But since the symptoms may recur, medication may need to continue long-term.

References

Article

  1. Guillevin L, Cohen P, Gayraud M, et al. Churg-Strauss syndrome. Clinical study and long-term follow-up of 96 patients. Medicine (Baltimore). Jan 1999;78(1):26-37.
  2. Choi YH, Im JG, Han BK, Kim JH, Lee KY, Myoung NH. Thoracic manifestation of Churg-Strauss syndrome: radiologic and clinical findings. Chest. Jan 2000;117(1):117-24.
  3. Churg J, Strauss L. Allergic granulomatosis, allergic angiitis, and periarteritis nodosa. Am J Pathol. Mar-Apr 1951;27(2):277-301.
  4. Hellmich B, Ehlers S, Csernok E, Gross WL. Update on the pathogenesis of Churg-Strauss syndrome. Clin Exp Rheumatol. Nov-Dec 2003;21(6 Suppl 32):S69-77.
  5. Masi AT, Hunder GG, Lie JT, et al. The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis). Arthritis Rheum. Aug 1990;33(8):1094-100.
  6. Schmitt, W. H., Linder, R., Reinhold-Keller, E. and Gross, W. L. (2001), Improved differentiation between Churg-Strauss syndrome and Wegener's granulomatosis by an artificial neural network. Arthritis & Rheumatism, 44: 1887–1896.
  7. Franco J, Artes MJ. Pulmonary eosinophilia associated with montelukast. Thorax. Jun 1999;54(6):558-60.
  8. Tsurikisawa, N., Taniguchi, M., Suzuki, S. and Akiyama, K. (2003), Effects of a nitro compound patch on neuropathy in Churg-Strauss syndrome. Allergy, 58: 686–687.
  9. Jennette JC, Falk RJ, Andrassy K, et al. Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum. Feb 1994;37(2):187-92.

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Last updated: 2019-07-11 20:53