Korean J Crit Care Med.  2015 Aug;30(3):212-217. 10.4266/kjccm.2015.30.3.212.

Successful Immunoglobulin Treatment in Severe Cryptogenic Organizing Pneumonia Caused by Dermatomyositis

• Lee DH
• Yeo JH
• Kim YI
• Gim SJ
• Sohn JW
• Yhi JY

• KMID: 2227652
• DOI: http://doi.org/10.4266/kjccm.2015.30.3.212

Abstract

In connective tissue diseases, autoantibodies cause pulmonary interstitial inflammation and fibrosis, and patients require treatment with an immunosuppressive agent such as a steroid. Dermatomyositis is an incurable, uncommon form of connective tissue disease that occasionally causes diffuse pulmonary inflammation leading to acute severe respiratory failure. In such cases, the prognosis is very poor despite treatment with high-dose steroid. In the present case, a 46-year-old man was admitted to our hospital with dyspnea. He was diagnosed with dermatomyositis combined with cryptogenic organizing pneumonia (COP) with respiratory failure and underwent treatment with steroid and an immunosuppressive agent, but the COP was not improved. However, the respiratory failure did improve after treatment with intravenous immunoglobulin, which therefore can be considered a treatment option in cases where steroids and immunosuppressive agents are ineffective.

Keyword

cryptogenic organizing pneumonia; dermatomyositis; immunoglobulins; lung diseases, interstitial

MeSH Terms

Autoantibodies
Connective Tissue Diseases
Cryptogenic Organizing Pneumonia*
Dermatomyositis*
Dyspnea
Fibrosis
Humans
Immunoglobulins*
Immunosuppressive Agents
Inflammation
Lung Diseases, Interstitial
Middle Aged
Pneumonia
Prognosis
Respiratory Insufficiency
Steroids
Autoantibodies
Immunoglobulins
Immunosuppressive Agents
Steroids

Figure

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  Fig. 1. Chest X-ray. Chest X-ray on the day of admission, showing an ill-defined consolidation in both lower lobes and infiltration in the peripheral lung in both middle lung fields (A). Chest X-ray on day 22 of hospitalization, showing no improvement of the infiltration in either lower lobe (B). Chest X-ray on day 25 of hospitalization, after administration of intravenous immunoglobulin, showing improvement of the infiltration in both lower lobes (C).

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  Fig. 2. Chest computed tomography (CT). Chest CT at admission, showing peripheral consolidations in both lungs with ground-glass opacity mainly in the dependent portion (A). Chest CT on day 15 of hospitalization, showing worsening of the peripheral consolidation in both lower lungs, as well as bronchovascular thickening (B). Chest CT on day 31 of hospitalization, showing improvement of the organizing pneumonia (C). The severity of consolidation that had been dominant in the peripheral and dependent portions of both lungs was reduced.

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  Fig. 3. Magnetic resonance imaging of the femur, showing a subtle signal increase and enhancement in the vastus lateralis, vastus intermedius, rectus femoris, and adductor muscles with vascular engorgement.

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  Fig. 4. Bronchoscopic biopsy showing lymphoplasmacytic infiltration in the interstitium, and a young fibroblastic nodule.

Reference

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