대한흉부영상의학회 Korean Society of Thoracic Radiology

Discussion

Diagnosis With Brief Discussion

Diagnosis

Desquamative interstitial pneumonia

Radiologic Findings

Fig 1. Chest PA shows multifocal mass like consolidation at right lung field.
Fig 2-6. CT scans reveals multiple patchy or ill-defined nodular GGA, somewhat peripheral predominant, nonsegmental opacities in both lungs.

Pathology :
Lung, right upper lobe, wedge resection
: Consistent with desquamative interstitial pneumonia

Brief Review

DIP as pulmonary manifestation of autoimmune disease:
A review of 24 DIP cases (Japan) indicates that DIP may be associated with a high incidence of autoimmune disorders.
Mild to moderate increases in IgG were seen in 59% and IgE in 45%. Interestingly, positive findings for some kind of autoantibodies (e.g. ANA, RF, ribonucleoprotein, SS-A, centromere) or high serum levels of Ig G (1,700-4,440 mg/mL) were observed in 19 cases (79%) by our re-viewing 24 pathologically definite DIP cases. The association of organ-specific immunological diseases, elevated ANA, IgG and IgE levels and BAL eosinophilia/neutrophilia in DIP suggests that the pathogenesis is immunologically mediated with the participation of a type I allergic reaction to some kind of exogenous dust including cigarette smoke, or an immunologically altered state such as an autoimmune disorder. Other study reported, DIP may be one of pulmonary manifestations of inflammatory bowel disease.
(Our case, the test for autoimmune Ab or Ig was not done)

DIP is an uncommon form of interstitial pneumonia that occurs frequently in patients between 30 and 50 years of age. Approximately 90% of patients with DIP are cigarette smokers, but can occurs in nonsmokers (10~42%). The most common radiographic appearance of DIP is bilateral basal hazy increased opacification with a reduction in lung volumes. However, the radiographs are normal in about 5% to 20% of patients who have biopsy-proven DIP. The predominant HRCT abnormality is also bilateral area of ground-glass attenuation, reflecting the filling of alveolar airspaces by macrophages. A subpleural and basal predominance is often present. Although reticular opacity may be associated with ground-glass attenuation, honeycombing is uncommon. Of cases, 32%-75% develop small cystic airspaces within areas of ground-glass attenuation. Histopathologic correlation suggests that some of these cysts represent bronchioliectasis and dilated alveolar ducts, without honeycomb fibrosis. Because of its association with cigarette smoking, centrilobular emphysema is also common present. Spontaneous improvement may occur in untreated patients. The response to corticosteroid is unclear; however, most patients report a symptomatic improvement.

Please refer to

Case 4, Case 266, Case 615, Case 620, Case 840, Case 1127,

KSTR Imaging Conference 2004 Spring  Case 8 ,
KSTR Imaging conference 2010 Summer  Case 19 ,
KSTR Imaging Conference 2011 Spring  Case 6,

References

1. Ishii H, Iwata A, Sakamoto N, Mizunoe S, Mukae H, Kadota J. Desquamative interstitial pneumonia (DIP) in a patient with rheumatoid arthritis: is DIP associated with autoimmune disorders?. Intern Med. 2009;48(10):827-30.
2. Kawabata Y, Takemura T, Hebisawa A, et al. Eosinophilia in bron-choalveolar lavage fluid and architectural destruction are featuresof desquamative interstitial pneumonia. Histopathology52: 194-202, 2008
3. Sebastian M, Wojciech P, Pulmonary manifestations of inflammatory bowel disease. Arch Med Sci. 2015 Dec 10; 11(6): 1179

Keywords

lung, interstitial lung disease, desquamative interstitial pneumonia,