J Korean Rheum Assoc.  2010 Dec;17(4):448-453. 10.4078/jkra.2010.17.4.448.

A Case of Rituximab Treatment for Interstitial Lung Disease in a Patient with Antisynthetase Syndrome

• Jo YM
• Jung JK
• Kim YJ
• Lee SY
• Lee SW
• Chung WT

• KMID: 2201907
• DOI: http://doi.org/10.4078/jkra.2010.17.4.448

Abstract

The clinical manifestations of antisynthetase syndrome are severe interstitial pneumonitis, mild polyarthritis, and myositis. This disease is accompanied by anti-Jo-1 antibodies and anti-Ro/SSA antibodies and occasionally by the concurrence of anti-Jo-1 and anti-Ro/SSA antibodies, which leads to a more severe form of interstitial lung disease. In this case, the patient was transferred to our hospital because of pulmonary fibrosis with myositis and diagnosed with antisynthetase syndrome and the concurrence of anti-Jo-1 with anti-Ro/SSA antibodies. He was refractory to glucocorticoids, and developed leucopenia and thrombocytopenia. He was treated with rituximab infusions, but the interstitial pneumonitis progressed very rapidly and he died.

Keyword

Antisynthetase syndrome; Rituximab; Interstitial pneumonitis

MeSH Terms

Antibodies
Antibodies, Monoclonal, Murine-Derived
Arthritis
Glucocorticoids
Humans
Lung Diseases, Interstitial
Myositis
Pulmonary Fibrosis
Rituximab
Thrombocytopenia
Antibodies
Antibodies, Monoclonal, Murine-Derived
Glucocorticoids
Myositis

Figure

• 

  Fig. 1. Pulmonary function test showed restrictive pattern and reduced diffusion capacity (forced vital capacity [FVC], 46%: forced expiratory volume in 1 sec [FEV1], 53%: CO diffusing capacity [DLCO], 44%).

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  Fig. 2. Chest radiological findings showed bilateral interstitial pulmonary fibrosis.

• 

  Fig. 3. Initial high resolution computed tomography scan of the thorax showed fibrosis in the peripheral lung fields, with bronchiectasis and diffuse ground glass opacity consistent with a pattern of non-specific interstitial pneumonitis, or bronchiolitis obliterans organizing pneumonia.

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  Fig. 4. Repeated (10 days later) high resolution computed tomography scan showed an increase in the ground glass opacity, aggravation of the fibrotic changes, and bronchial wall thickening.

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