An Abdominal Presentation of Churg-Strauss Syndrome

Churg-Strauss syndrome is a small and medium vessel vasculitis that is also known as allergic granulomatous angiitis. It most commonly presents with an asthma like symptoms. It was first described in Mount Siani Hospital, New York in 1951 by Jacob Churg and Lotte Stauss and was recognised after the study of a series of 13 patients who had asthma, eosinophilia, granulomatous inflammation necrotising systemic vasculitis and necrotising glomerulonephritis. We describe a case of Churg-Strauss syndrome presenting with abdominal pain and later during the hospital admission a mono-neuritis multiplex syndrome affecting the lower limbs. The patient presented in such an atypical fashion with abdominal signs and symptoms that they required laparotomy and the diagnosis was made after histological examination of tissue taken at the time of surgery. Treatment with immunosuppression and aggressive rehabilitation achieved a progressive recovery which continued on discharge from hospital.

Ileal Ulcers and Cytomegalovirus Infection in a Case of Churg-Strauss Syndrome

Churg-Strauss syndrome, or allergic granulomatous angiitis, is an uncommon vasculitic syndrome. We describe a 53-year-old man with Churg-Strauss syndrome and subsequent opportunistic cytomegalovirus enterocolitis. During intensive care, including steroid-pulse therapy, the patient developed rapidly progressive anemia caused by active bleeding from his small intestine, resulting in resection of 20 cm of ileum. Diagnosis of Churg-Strauss syndrome was confirmed both by characteristic clinical features and by histology. Histologic examination also revealed multiple shallow ulcers accompanied by cytomegalovirus infection. Characteristic angiitis was found in the ileum with normal-like mucosa, and it was not necessarily associated with ileal ulcers. This finding suggests that cytomegalovirus infection may be one of the causes or exacerbating factors for ileal ulcers in Churg-Strauss syndrome, although ulcers of the intestine have usually been considered to be caused by ischemia resulting from angiitis.

Allergic granulomatous angiitis

Allergic granulomatous angiitis (AGA) - Churg-Strauss syndrome, is a rare autoimmune disease characterized by three distinct clinical phases prodromal, eosinophilic, and vasculitic, and most of respiratory symptoms and signs begin in the first two phases of the disease. Two female patients of different age, who fulfilled the diagnostic criteria for AGA, and were in different phases and with the different duration of the disease are presented. The first patient (24 years of age) was admitted to the hospital due to aggravation of asthma, heart failure, and polyneuropathy. The second one (45 years of age) was also hospitalized due to the worsening of asthma polyneuropathy, and fever. Both were treated continuously with glucocorticoids. The older patient also received a total of six pulse doses of cyclophosphamide. Satisfactory response to such a treatment was achieved in both cases.