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What is allergic angiitis and granulomatosis?

Allergic angiitis and granulomatosis (AAG, also known as Churg-Strauss syndrome) is a rare disorder characterized by inflammation of blood vessels (vasculitis). The disorder can affect multiple organ systems, especially the respiratory system. Other significant characteristics of the disorder include the development of inflammatory nodular tissues called granulomas (granulomatosis) and abnormal clustering of certain white blood cells (hypereosinophilia) in the blood and the tissues. In medical terms, this disorder is also called eosinophilic granulomatosis with polyangiitis.

What are its main signs and symptoms?

Mainly, this disease affects the arteries in the body, hence, depending on the organs affected and the degree of involvement, the symptoms may vary. Though symptoms may vary, blood eosinophilia, asthma, and/or nasal sinus polyps are encountered in almost all patients. Some of the other symptoms include:

  • Fever and fatigue
  • Unusual weakness in the hands or feet
  • Pain in the abdomen, muscle, and/or joint
  • Chest pain or palpitations (heartbeat, which can be irregular)
  • Sudden, severe weight loss
  • Skin rash (recurrent and diffuse hives, purpura, or subcutaneous nodules)
  • Numbness or tingling sensation in the hands or feet
  • Increasing shortness of breath or coughing which does not improve with medications
  • Phlebitis (inflammation of a vein)
  • Pulmonary embolism (blockage in one of the arteries in the lungs, most commonly, due to a blood clot)
  • Blood in the stools

What are the main causes?

The main cause of this condition remains unknown yet. However, some of the potential causes are:

  • Environmental factors
  • Genetics
  • Immunological
  • Auto-immune conditions like anti-Neutrophil Cytoplasmic Antibodies (ANCA)- positivity
  • Hormone-like chemicals in the blood (cytokines)

How is it diagnosed and treated?

The diagnosis of allergic angiitis and granulomatosis is made by the physician after considering all the signs and symptoms and conducting a thorough physical examination. Depending on this, the physician may also advice certain tests which include:

  • Blood tests
  • Specialized imaging studies which include chest X-rays
  • Sometimes, a biopsy may be performed of an affected tissue or organ which will help to diagnose typical features of this condition
  • Blood test to measure the level of antineutrophil cytoplasmic autoantibodies (ANCA)
  • Bronchoscopic lavage
  • Heart function tests like 2D Echocardiogram
  • Lung function tests

Treatment:

The treatment for this condition depends on its severity:

  • Individuals in whom the manifestations of vasculitis are not severe (no vasculitis related digestive, cardiac, cerebral or kidney involvement) corticosteroids can be used for treatment. With this treatment, there might be a relapse in one-third of the patients, whereas 90% of the patients may have complete remission.
  • In individuals with severe manifestations, a combination of corticosteroids and an immunosuppressant drug (such as azathioprine, cyclophosphamide, or methotrexate) is used. Generally, for the first three to six months, a combination of corticosteroids and cyclophosphamide are used, followed by switching of cyclophosphamide with methotrexate or azathioprine for maintenance for several additional months.
  1. Medicines for Allergic Angiitis and Granulomatosis

Medicines for Allergic Angiitis and Granulomatosis

Medicines listed below are available for Allergic Angiitis and Granulomatosis. Please note that you should not take any medicines without doctor consultation. Taking any medicine without doctor's consultation can cause serious problems.

Medicine NamePack SizePrice (Rs.)
KolqKolq Capsule28.0
EkonEkon 10 Mg Tablet14.0
AllercetAllercet 10 Mg Tablet12.0
ActAct 5 Mg/60 Mg Tablet26.0
CetezeCETEZE 10MG TABLET 10S0.0
Alday AmAlday Am 5 Mg/60 Mg Tablet26.0
Ceticad PlusCeticad Plus Tablet4.0
AmbcetAmbcet 5 Mg/30 Mg Syrup32.0
CetipenCetipen Tablet1.0
Ambcet ColdAmbcet Cold 5 Mg/60 Mg Tablet39.0
Cetiriz GCetiriz G Syrup13.0
Broncocet AmBroncocet Am Syrup33.0
CetizCETIZ 10MG TABLET 10S0.0
Car CaCar Ca Syrup29.0
CetmakCetmak 10 Mg Tablet0.0
Car OdCar Od Tablet0.0
CetnirCetnir Syrup8.0
CetaxolCetaxol Tablet28.0
Cet PCet P Syrup31.0
Cetzine ACetzine A 5 Mg/60 Mg Tablet35.0
CetractCetract 10 Mg Tablet16.0
Levolyte ALevolyte A 5 Mg/60 Mg Tablet32.0
CetrahistCetrahist 10 Mg Tablet21.0
Onimex CzOnimex Cz 2.5 Mg/30 Mg Syrup48.0

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References

  1. Jessurun J et al. Allergic angiitis and granulomatosis (Churg-Strauss syndrome): report of a case with massive thymic involvement in a nonasthmatic patient.. Hum Pathol. 1986 Jun;17(6):637-9. PMID: 3710473
  2. Jacob Churg, Lotte Strauss. Allergic Granulomatosis, Allergic Angiitis, and Periarteritis Nodosa. Am J Pathol. 1951 Apr; 27(2): 277–301. PMID: 14819261
  3. C C Chow et al. Allergic granulomatosis and angiitis (Churg-Strauss syndrome): response to 'pulse' intravenous cyclophosphamide.. Ann Rheum Dis. 1989 Jul; 48(7): 605–608. PMID: 2774702
  4. Orphanet. Eosinophilic granulomatosis with polyangiitis. French National Institute for Health and Medical Research. [internet]
  5. Thomas F. Allergic granutomatous angiitis (Churg-Strauss syndrome) . The Journal of Allergy and Clinical Immunology; Elsevier. [internet]
  6. Thomas F. Allergic granutomatous angiitis (Churg-Strauss syndrome) . The Journal of Allergy and Clinical Immunology; Elsevier. [internet]
  7. F Lhot. Allergic angiitis with granulomatosis: the Churg and Strauss syndrome.  La Revue de Médecine Interne 15 Suppl 2:226s-233s · February 1994
  8. Alfonse T Masi et al. [text]. American College of Rheumatology. [internet]
  9. Vasculitis Patient Powered Reserch Network. [internet]. Eosinophilic Granulomatosis with Polyangiitis

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