PURPOSE: The purpose of this study was to evaluate the radiological, pathologic and serological patterns in patients with Polymyositis-Dermatomyositis [PMD] associated interstitial lung disease at our institution.
METHODS: All patients admitted to our facility with a diagnosis of Polymyositis-Dermatomyositis during a seven year period, from 12/10/1997 to 05/13/04 had their records reviewed for serologic, radiographic as well as pathological data.40 patients with a primary diagnosis of PMD were seen during this period. Of these 20 had CT imaging and/or lung biopsies performed [1 Transbronchial biopsy and 3 Open lung biopsies].7 patients were finally identified as having interstitial lung disease.
RESULTS: The 7 patients ranged in age from 29 to 73 years.4 of the seven [57.1%] had a Usual Interstitial pneumonia[UIP] pattern while 2 patients [28.6%] had the Non Specific Interstitial pneumonia[NSIP] pattern. In 1 patient [no lung biopsy done], a definitive pattern could not be discerned. Antinuclear Antibodies [ANA] were present in 1 of 3 patients tested [33%]; Anti JO-1 antibodies were positive in 4 of 5 patients tested [80%]Serum Aldolase levels were high in 4 of 4 patients tested [100%].
CONCLUSION: In contrast to other studies the UIP pattern appeared to be the dominant pattern of interstitial lung disease in our patients. It is not clear why this was the case. Taking into consideration the reported better prognosis of the NSIP pattern we suggest that more studies be done to better understand the above observations.Neither the UIP nor the NSIP pattern appeared to be related to the presence or absence of Anti JO-1 antibodies, nor to the magnitude of the antibody when present.
CLINICAL IMPLICATIONS: NSIP might not be the dominant pathologic pattern seen in Interstitial Lung Disease associated with Polymyositis-Dermatomyositis as previously reported.Further studies are needed.
DISCLOSURE: Sebastine Elendu, None.