A 53 Year Old Man With A Long History Of Asthma Presents With Blood In Urine

A 53 year old man with a history of asthma and allergic rhinitis was admitted to the hospital with acute breathlessness, cough and presence of blood in urine. On examination , he appears pale and unwell. His pulse is 90 beats/ minute and Blood pressure 160/90 mm Hg. Abdominal and chest examination did not reveal any significant abnormalities. Urinalysis shows ++ for blood and protein. Complete blood count shows Hb = 7 g/dl, WBC = 11000/dl with a high percentage of eosinophils, increased ESR and slightly raised urea and creatinine.  A biopsy was taken and shows the following histologic picture:

An eosinophillic vasculitis

What is the diagnosis?
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Churg- Strauss Syndrome

Case Discussion:
Churg- Strauss Syndrome which is also known as Eosinophillic granulomatosis is a rare autoimmune condition that affects small and medium sized arteries and veins.

American College of Rheumatology proposed the following six criteria for the diagnosis of Churg- Strauss Syndrome.

1. Asthma (with wheezing and/or expiratory ronchi)
2. Eosinophilia of more than 10% in the peripheral blood. 
3. Paranasal sinusitis
4. Pulmonary infiltrates (may be transient)
5. Histologic proof of vasculitis with extravascular eosinophils.
6. Polyneuropathy or mononeuritis multiplex.

The presence of four or more criteria yeilds a sensitivity of 85% and specificity of 99.7% for the diagnosis of Churg- Strauss Syndrome.

Clinical Features: It is a very variable illness with some patients having minimal or no symptoms while others may have life threatening complications. The disease is characterized by therr stages. they include:

1. Allergic Stage: usually the first stage of Chrug-Strauss syndrome marked by allergic reactions including asthma, allergic rhinitis and sinusitis.

2. Eosinophillic stage: Eosinophils normally constituent a small percentage of the white cell count but in Chrug- Strauss syndrom markedly high number of eosinophils are seen in the blood and the tissues that may cause significant damage and symptoms may include:

• Fever
• Weight loss
• Decreased appetite
• Asthma exacerbation.
• Abdominal pain
• GI bleeding

3. Vasculitic Stage: is characterized by severe blood vessel inflammation, that may cause reduced blood flow through out the body including the skin, heart, muscle, bone, digestive tract as well as kidneys. 

Treatment: There is no cure for Chrugg-Strauss syndrome but the symptoms could be controlled by glucorticoid drugs like prednisolone and immunosuppresive drugs including azathioprine and cyclophosphamide.