J Rheum Dis.  2014 Feb;21(1):25-29. 10.4078/jrd.2014.21.1.25.

Acute Polymyositis/systemic Lupus Erythematosus Overlap Syndrome with Severe Subcutaneous Edema and Interstitial Lung Disease

• Jin UR
• Kwack KS
• Park KJ
• Kwon JE
• Kim SY
• Kim KC
• Ban GY
• Jung JY
• Suh CH
• Kim HA

• KMID: 2222985
• DOI: http://doi.org/10.4078/jrd.2014.21.1.25

Abstract

Inflammatory myopathy is characterized by symmetrical proximal muscle weakness, elevated muscle enzyme levels and favorable response to glucocorticoids therapy. Although periorbital edema is a common manifestation of inflammatory myopathy, generalized subcutaneous edema is very rare. We report here a case of a 47-year-old female patient with acute polymyositis/systemic lupus erythematosus overlap syndrome with generalized subcutaneous edema and interstitial lung disease. We aggressively treated the disease with high-dose glucocorticoids, intravenous immunoglobulin, and immunosuppressive agents.

Keyword

Inflammatory myopathy; Overlap syndrome; Subcutaneous edema; Interstitial lung disease

MeSH Terms

Edema*
Female
Glucocorticoids
Humans
Immunoglobulins
Immunosuppressive Agents
Lung Diseases, Interstitial*
Middle Aged
Muscle Weakness
Muscles
Myositis
Glucocorticoids
Immunoglobulins
Immunosuppressive Agents

Figure

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  Figure 1. Gross appearance of generalized edema of upper (A) and lower extremities (B) in a 47-year-old woman with acute polymyositis.

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  Figure 2. Radiologic findings of computed tomography (CT) scan of the chest. (A) The CT scan of the chest upon admission shows interstitial lung disease with consolidation and ground-glass opacities in peripheral lungs, bilateral pleural effusion, and minimal pericardial effusion. (B) The resolution of the interstitial lung disease after treatment.

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  Figure 3. Radiologic findings of magnetic resonance imaging (MRI) of the thigh. (A) Axial T1-weighted image only shows generalized subcutaneous edema with focal low-signal subcutaneous fluid adjacent to vastus lateralis muscle (arrows). (B) Axial fat-suppressed fast spin-echo T2-weighted image shows a pattern of patchy elevated signal within multiple muscles in all three compartments. Biceps femoris, semimembranosus, and adductor magnus are relatively spared. (C) Axial fat-suppressed contrast-enhanced T1-weighted image shows a similar pattern of elevated signal within the affected muscles similar to the distribution seen on T2-weighted images. There is patchy enhancement of the quadriceps femoris group, sartorius, gracilis, and semitendinosus muscles (arrows).

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  Figure 4. Biopsy specimen of left deltoid muscle revealing interstitial and perifascular inflammatory cell infiltrates, degenerating fibers, perivascular inflammatory cell infiltrates and interstitial fibrosis consistent with polymyositis (H&E, ×200).

Reference

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