Churg-Strauss vasculitis

=Churg-Strauss Vasculitis = Churg-Strauss vasculitis, also known as eosinophilic granulomatosis with polyangiitis (EGPA), is a rare autoimmune condition characterized by inflammation of small to medium-sized blood vessels. This condition is part of a group of diseases known as ANCA-associated vasculitides.

History

Churg-Strauss vasculitis was first described in 1951 by Drs. Jacob Churg and Lotte Strauss. They identified the disease as a distinct form of vasculitis associated with asthma and eosinophilia.

Epidemiology

The disease is rare, with an estimated incidence of 1-3 cases per million people per year. It typically affects adults between the ages of 30 and 50 and is slightly more common in males than females.

Pathophysiology

Churg-Strauss vasculitis is characterized by three distinct phases:

• Allergic phase: This phase is marked by asthma and allergic rhinitis.
• Eosinophilic phase: This phase involves peripheral blood eosinophilia and eosinophilic infiltration of tissues.
• Vasculitic phase: This phase is characterized by systemic vasculitis affecting multiple organs.

The exact cause of Churg-Strauss vasculitis is unknown, but it is believed to involve a combination of genetic and environmental factors. The presence of anti-neutrophil cytoplasmic antibodies is noted in some patients.

Clinical Features

The clinical presentation of Churg-Strauss vasculitis can vary widely, but common symptoms include:

• Asthma
• Sinusitis
• Skin rashes
• Peripheral neuropathy
• Gastrointestinal symptoms
• Cardiac involvement

Diagnosis

Diagnosis is based on clinical criteria, laboratory tests, and histopathological findings. Key diagnostic criteria include:

• Asthma
• Eosinophilia (>10% of white blood cells)
• Neuropathy
• Pulmonary infiltrates
• Paranasal sinus abnormalities
• Biopsy showing eosinophilic vasculitis

Treatment

Treatment typically involves the use of corticosteroids and immunosuppressive agents such as cyclophosphamide or methotrexate. The goal is to control inflammation and prevent organ damage.

Prognosis

With appropriate treatment, the prognosis for patients with Churg-Strauss vasculitis has improved significantly. However, the disease can be life-threatening if not treated promptly.

See Also

• Vasculitis
• Eosinophilia
• Asthma
• Churg, J., & Strauss, L. (1951). Allergic granulomatosis, allergic angiitis, and periarteritis nodosa. American Journal of Pathology, 27(2), 277-301.
• Mahr, A., et al. (2006). Prevalences of polyarteritis nodosa, microscopic polyangiitis, Wegener's granulomatosis, and Churg-Strauss syndrome in a French urban multiethnic population in 2000: a capture-recapture estimate. Arthritis & Rheumatism, 54(1), 92-99.

NIH genetic and rare disease info

• NIH info on Churg-Strauss vasculitis

Churg-Strauss vasculitis is a rare disease.