Tuberc Respir Dis.  2008 Oct;65(4):323-327.

Systemic Lupus Erythematosus Associated with Interstitial Pneumonia and Achalasia

• Kwon HL
• Hong KW
• Lim SJ
• Park SY
• Bae YD
• Kim KH
• Choi JH
• Mo EK
• Park YB

Abstract

Systemic lupus erythematosus (SLE) is a multisystem disorder where the etiology is not clearly known. Symptomatic chronic interstitial pneumonitis is an uncommon manifestation, with a reported prevalence of 3~13%. Achalasia is rare disease that presents with failure in the relaxation of the esophagus sphincter. A 22-year-old woman was admitted to our hospital because of fever, cough and dyspnea. The patient had a history of pericardial effusion and Raynaud's phenomenon. The results of laboratory tests indicated the presence of lymphopenia and included positive antibody tests for antinuclear antibody and anti Sm antibody. A chest X-ray demonstrated the presence of peribronchial infiltration on both lung fields. A Chest CT image showed interlobar septal thickening, ground-glass opacity and a honeycomb appearance in both lung fields and esophageal dilatation with air fluid level. An esophagogram showed the presence of dilated esophagus ends that represented the non-relaxed lower esophageal sphincter. Manometry demonstrated incomplete sphincter relaxation. The case was diagnosed as systemic lupus erythematosus associated with interstitial pneumonia and achalasia.

Keyword

Interstitial pneumonia; Achalasia; SLE

MeSH Terms

Antibodies, Antinuclear
Cough
Dilatation
Dyspnea
Esophageal Achalasia
Esophageal Sphincter, Lower
Esophagus
Female
Fever
Humans
Lung
Lung Diseases, Interstitial
Lupus Erythematosus, Systemic
Lymphopenia
Manometry
Pericardial Effusion
Prevalence
Rare Diseases
Relaxation
Thorax
Young Adult
Antibodies, Antinuclear

Figure

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  Figure 1 Chest PA shows diffuse reticularities with peribronchial infiltration on both lower lung fields.

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  Figure 2 HRCT scan shows interlobular septal thickening, ground glass opacity and honeycomb appearance on right upper lung and both lower lungs.

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  Figure 3 (A) Chest CT shows dilated middle and lower esophagus which filled with food. (B) Barium esophagography shows that lower esophageal sphincter dose not relax normally and the esophageal body undergoes nonperistaltic contraction.

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