Five Things All Internists Should Know About ILD
We learned so many pearls about ILD at the noon conference presented by Dr. Jared Chiarchiaro today!
#1 Not all septal lines/reticular opacities are on CT chest are fibrosis, consider differentials
Ddx for septal lines/reticulation: pulmonary edema, lymphagetic carcinomatosis, pulmonary hemorrhage, atelectasis, etc (even radiologist sometimes call it wrong!)
#2 CT chest maybe diagnostic of IPF with subpleraul basal reticular opacities and honeycombing in the absence of other etiologies
IPF Imaging Criteria
Septal lines - reticular, subpleural, lower lobe
Honeycombing *Features: stacking up, shared walls (in contrast with cystic disease which does not have shared walls)
With or without traction bronchiectasis
Absence of other features: extensive GGO (ie, IPF is featured by “paucity of GGOs”), nodules, cyst
Exclusion of other potential etiologies: CTDs, meds (eg, nitrofuratoin), occupational/environmental, smoking-related lung disease
#3 Consider anti-fibrotic meds in IPF with declining PVC
Nintedanib (Richeldi L et al. NEJM 2014) - taken TID, causes early satiety, photosensitivity
Pirfenidone (King TE et al. NEJM 2014) - taken BID, causes diarrhea (even with loperamide)
Fortunately, many patients have stable disease
Unfortunately, we cannot predict who will decline
#4 Patient with ILD that require further evaluation
Women, particularly <50 yo
Extrathoracic disease
History: rash, triphasic Raynaud’s, inflammatory arthritis, sicca symptoms, muscle weakness
Exam: skin, mechanic’s hands, Gottron papules, sclerodactyly, digital ulcers, synovitis, muscle weakness
About CTD and ILD
ILD often identified in the setting of an already established CTD (SSc, RA, PM, pSS)
ILD may be the first manifestation of CTD (anti-synthetase syndrome)
Some have ILD with autoimmune features (meet some but not all dx criteria for CTD)
CTD-ILD prognosis is better than IPF (RA with UIP is an exception)
Serologies: send these labs (false positives are common)
ANA > 1:320 or nucleolar pattern at any titer
RF > 3x upper limit
Myositis panel (70% of anti-synthetase syndrome present with ILD and has a negative ANA)
#5 Prognosis discussion: Ask your patients what they know, tell them the diagnosis, ask them to choose a surrogate