• Title/Summary/Keyword: Mononeuritis multiplex

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An overlap syndrome of Churg-Strauss syndrome and rheumatoid arthritis

  • Bae, Seung Il;Jang, Jong Geol;Kim, Hun Tae;Ahn, Hee Yun;Kim, Min Jung;Kim, Hyun Je;Lee, Choong Ki;Hong, Young Hoon
    • Journal of Yeungnam Medical Science
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    • v.32 no.2
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    • pp.127-131
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    • 2015
  • Churg-Strauss syndrome (CSS) is a necrotizing vasculitis with extra-, peri-vascular eosinophilic infiltration. Chronic symmetric polyarthritis with the presence of rheumatoid factor (RF) and anti-cyclic citrullinated peptide antibody are the mainstay of rheumatoid arthritis (RA) diagnosis. Mononeuritis multiplex is a peripheral neuropathy involving more than 2 separate nerve areas. A 62-year-old male patient was referred for left foot drop and polyarthritis of both hands and feet for 4 months. During evaluation, mononeuritis multiplex was detected on nerve conduction study and electromyography tests: vasculitis with neutrophil, eosinophil, and lymphocyte infiltration on peroneal nerve biopsy. A positive response to methacholin and bronchodilator was observed on the pulmonary function test. Radiologic tests showed peri-articular soft tissue swelling and osteopenia on both hand and foot. Marked peripheral eosinophilia, high RF, and positive perinuclear anti-neutrophil cytoplasmic antibody were detected on blood tests. Here, we report on a patient with overlap syndrome of CSS and RA with review of the relevant literature, from which a few references to overlap syndrome of CSS and RA were available.

A Case of Churg-Strauss Syndrome with Interstinal Perforation (장천공이 동반된 Churg-Strauss 증후군 1예)

  • Park, Jung Hoon;Jung, Yun Seok;Kim, Yang Ki;Lee, Young Mok;Hwang, Jung Hwa;Kim, Ki-Up;Kim, Dong Won;Uh, Soo-Taek;Kim, Jae Jun
    • Tuberculosis and Respiratory Diseases
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    • v.66 no.5
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    • pp.374-379
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    • 2009
  • Churg-Strauss syndrome (CSS) or allergic granulomatous angiitis is a rare syndrome that is characterized by hypereosinophilic systemic necrotizing vasculitis affecting small- to medium-sized arteries and veins. In general, it occurs in individuals with pre-existing allergic asthma. When CSS appears in patients, it has the following characteristics: eosinophilia of more than 10% in peripheral blood, paranasal sinusitis, pulmonary infiltrates, histological proof of vasculitis with extravascular eosinophils, and mononeuritis multiplex or polyneuropathy. Therapeutic trials dedicated to Churg-Strauss syndrome have been limited due to the rarity of this disorder and the difficulty in making a histological diagnosis. Proper treatment of patients with CSS is not widely known. In this case study, we report on our experience with an unusual patient case, characterized by purpura and a perforation of the small intestine after inadequate steroid therapy.

A Case of Churg-Strauss Syndrome with Diffuse Alveolar Hemorrhage Presenting as Acute Acalculous Cholecystitis (미만성 폐포 출혈을 동반한 급성 담낭염으로 발현한 Churg-Strauss 증후군 1예)

  • Kim, Ji Eun;Kim, Ki Uk;Park, Hye-Kyung;Jeon, Doo Soo;Kim, Yun Sung;Lee, Min Ki;Park, Soon Kew
    • Tuberculosis and Respiratory Diseases
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    • v.66 no.3
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    • pp.225-229
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    • 2009
  • Churg-Strauss syndrome (CSS) is a disorder that is characterized by asthma, hypereosinophilia and systemic vasculitis affecting a number of organs. The manifestations of acute cholecystitis and diffuse alveolar hemorrhage are rarely reported in CSS. A 22-year-old woman with bronchial asthma visited our hospital complaining of right upper quadrant pain with a sudden onset. The abdominal computed tomography (CT) scan revealed gall bladder edema consistent with acute cholecystitis. On the initial evaluation, marked hypereosinophilia was observed in the peripheral blood smear. The nerve conduction velocity measurements and a skin biopsy performed to confirm the organ involvement of disease indicated typical mononeuritis multiplex and necrotizing vasculitis, respectively, which was complicated with CSS. On the 12th hospital day, ground glass opacity and consolidations were newly developed on both lung fields. The bronchoalveolar lavage (BAL) fluid showed increasing bloody return in sequential aliquots that were characteristic of a diffuse alveolar hemorrhage. We report a case of CSS with acute cholecystitis and diffuse alveolar hemorrhage.