140LU ILD End Stage

60F ongoing evaluation and management of connective tissue disease diagnosed 1 year ago

recently diagnosed CTD on the Sjogren’s/SLE spectrum (pruritic rash, sicca symptoms with compatible labial biopsy, Raynaud’s, abnormal nail capillaroscopy, ANA+, Ro >8.0, CCP+) complicated by ILD who presents to the ILD clinic

cutaneous findings, she reports the development of dyspnea on exertion which she first noticed over the summer of 9months ago

6 months agopositive ANA multiplex without specified titer
SSA > 8.0, remainder of ENA’s negative;
CRP 20.7 (ref < 5.0 mg/L),
SPEP mildly elevated alpha-2 and hypoalbuminemia,
negative tryptase.

CXR from 4 years ago

60F ILD question NSIP UIP 001 CXR 4years ago

 

CXR from 6months ago

60F ILD question NSIP UIP 002 CXR 4 mths ago

CT from 6months ago

1. Bibasilar reticular abnormalities with areas of intervening traction bronchiectasis and minimal ground-glass opacity but no honeycombing noted.
These findings are nonspecific but raise concern for interstitial fibrotic lung disease.
The imaging findings suggest a UIP versus NSIP pattern.
Clinical correlation isrecommended with any history of connective tissue disorder,occupational or domestic exposures or potential drug toxicity.
2. No lobar pneumonia, pneumothorax or pleural effusion.

 

Pathology

60F-ILD-question-NSIP-UIP-008 bx
  • The lung parenchyma is extensively fibrotic with fibrocystic changes. The interstitium shows only scant chronic inflammatory infiltrate, with a predominance of plasma cells. Fibroblastic foci are vanishingly rare. No granulomas or vasculitis are identified. Findings are consistent with end stage interstitial fibrosis or uncertainty etiology. The plasma cells suggest autoimmune disease.Pulmonary Function Trend Report

    Date FVC FEV1 ratio  FeF25-75  TLC
    3 months ago

    • FVC2.18 84%
    • FEV1  1.61 79%
    • ratio    74
    • TLC 3.38 74%
    • RV —
    • DLCO  8.01 41%
  • Current
    • FVC 2.31 89%
    • FEV1  1.76 86%
    • ratio    76
    • TLC —
    • RV–
    • DLCO   5.08 26%Assessment and Plan: