Churg strauss syndrome

CHURG STRAUSS SYNDROME

Churg Strauss syndrome is a form of vasculitis or inflammation of the blood vessels. In patients with a history of asthma or allergies, Churg-Strauss syndrome occurs in particular and features inflammation of the blood vessels in the lungs, skin, nerves, and abdomen. Small arteries and veins are the blood vessels that are affected in Churg-Strauss syndrome.

Churg-Strauss syndrome is rare.  The cause of the syndrome is uncertain, but it is associated with excessive over-activation of the immune system in individuals with underlying bronchospastic lung disease (asthma). However, the connection is not yet clear.

SYMPTOMS

Churg-Strauss syndrome differs widely from individual to individual. There are only mild symptoms in some people. Others develop serious life-threatening complications. 

The condition occurs in three stages, and grows increasingly worse. Asthma, chronic sinusitis, and elevated white blood cell count called eosinophils are present in almost anyone with the disease. Asthma starts typically five to nine years before the syndrome is diagnosed.

  • Fever
  • Weight loss
  • Sinus or nasal passage inflammation
  • Fatigue
  • Cough and shortness of breath
  • Chest pain
  • Skin lumps or nodules in the extremities
  • Diarrhea and abdominal pain
  • Inflamed bladder and or prostate gland
  • Numbness or weakness of the extremities is the result of nerve injury
  • Seizures and confusion (if the brain is affected)

DIAGNOSIS

Churg  Strauss  syndrome diagnosis is recommended when a patient with a history of asthma experiences the symptoms mentioned above. 

The doctor may observe lung, skin, and nerve defects during the examination. Blood pressure is checked to see if it is elevated and beyond normal. 

Blood testing of people with this syndrome typically indicates elevated levels of an uncommon white blood cell, called an eosinophil, and there are also elevated numbers of other white blood cells. Blood tests for kidney function and urinalysis may be abnormal when the kidneys are affected.

The chest x-ray image or CT scan of the chest will show areas of inflammation if the lungs are inflamed. 

A biopsy of the involved tissue, which reveals a distinctive inflammation pattern evident under a microscope, is the ultimate examination for diagnosis. An accumulated eosinophil is also seen in the abnormal tissue.

 

TREATMENT

There is no cure for Churg Strauss Syndrome, but the treatment of patients with this syndrome is aimed both at the immediate calming of blood vessel inflammation (vasculitis) and the suppression of the immune system. Treatment typically requires large doses of cortisone-related medicine (such as prednisone or prednisolone) to soothe inflammation and with cyclophosphamide (Cytoxan) for suppression of the active immune system.

Cyclophosphamide has traditionally been used in patients with Churg  Strauss   Syndrome; they are given in patients for a year or more. In a clinical study, patients with this syndrome did equally well if they were treated for 6 or 12 months with cyclophosphamide. This study indicates that doctors might now be able to prescribe Cytoxan for patients with this syndrome for a shorter (and thus less damaging) course.

Drug therapy will alleviate Churg Strauss Syndrome symptoms and bring the disease into remission. Yet relapses are frequent. 

Your doctor will also routinely perform blood tests and other tests to monitor your health and reaction to the medications you are taking.

Related Articles

TETRALOGY OF FALLOT

Overview and FactsTypes and SymptomsDiagnosis & MedicationsOverview and Facts Tetralogy of Fallot is a congenital heart defect that affects the [...]

TRICHINOSIS

Overview and FactsTypes and SymptomsDiagnosis & MedicationsOverview and Facts Trichinosis, also known as trichinellosis, is a parasitic infection caused by [...]

TRIGEMINAL NEURALGIA

Overview and FactsTypes and SymptomsDiagnosis & MedicationsOverview and Facts Trigeminal neuralgia is a neurological condition characterized by severe facial pain. [...]