Abstract
Introduction
Polymyositis/Dermatomyositis patients often have interstitial pneumonia (IP). Histology usually shows organising pneumonia (OP) and non-specific interstitial pneumonia (NSIP). They may present either to a pulmonologist (Group A) or a rheumatologist (Group B). Myositis-specific antibodies (MSA) could aid diagnosis in conjugation with clinical phenotype.
Aim
To compare clinical phenotypes and their MSA associations in patients with HRCT evidence of IP.
Methods
Clinical presentation and investigation results in each group were reviewed retrospectively.
Results
Of Group A [n=4]; 3/4 (75%) had a respiratory problem and 1/4 (25%) arthralgia as their first symptom. Of group B [n=7]; 4/7 (57%) of whom had myositis, 2/7 (29%) a respiratory problem and 1/7 (14%) arthralgia as their first symptom. MRI of the thighs showed myositis in 6/7 patients in Group B but was only performed in 1/4 patient in Group A and was negative. Anti-Jo1 was the most common antibody in Group B [5/7 (71%) vs 1/4 (25%)]. PL7 and PM-Scl were only seen in Group A.
Conclusion
MaIP presents with different phenotypes. Myositis is more common in those presenting to rheumatologists. Anti-Jo1 antibodies were most common in patients presenting to rheumatologists whilst PL-7 and PM-Scl were seen in those presenting to pulmonologists. Pulmonologists should be alert to the occurrence of MaIP without frank myositis.
- © 2013 ERS
