一個呼吸的距離

ILD in IM: anti-JO-1(+) -> 70% (anti-MDA5)

(IM: inflammatory myositis; Gottron's papules, mechanical hand )

Elevated RF in RA -> survey extra-articular minifestation, eg. lung

Scleroderma: Scl-70(+)-> ↑lung involvement (Tx: MMF, Rituximab)

診斷時，要看 (1)Clinical domain (2) Serologic domain (3)Morphologic domain這三樣

建議抽的血:

B. Serologic domain

1. ANA ⩾1:320 titre, diffuse, speckled, homogeneous patterns or a. ANA nucleolar pattern (any titre) or b. ANA centromere pattern (any titre)

2. Rheumatoid factor ⩾2× upper limit of normal

3. Anti-CCP

4. Anti-dsDNA

5. Anti-Ro (SS-A)

6. Anti-La (SS-B)

7. Anti-ribonucleoprotein

8. Anti-Smith

9. Anti-topoisomerase (Scl-70)

10. Anti-tRNA synthetase (e.g. Jo-1, PL-7, PL-12; others are: EJ, OJ, KS, Zo, tRS)

11. Anti-PM-Scl

12. Anti-MDA-5

參考文獻:

https://www.thoracic.org/statements/resources/interstitial-lung-disease/interstitial-pna-autoimmune.pdf

IPAF: 灰色地帶，還是要想到有autoimmune disease的可能。

IPF藥物的調整

Panel: Therapies identified in clinical trials as harmful, ineffective, or effective in the treatment of idiopathic pulmonary fibrosis

Potentially harmful therapies • Ambrisentan81 • Everolimus82 • Prednisolone, azathioprine, acetylcysteine9 • Warfarin83

Potentially ineffective therapies • Bosentan84 • Imatinib85 • Macitentan86 • Acetylcysteine87 • Sildenafil88

Effective disease-modifying therapies • Nintedanib89 • Pirfenidone90,91

http://www.thelancet.com/pdfs/journals/lancet/PIIS0140-6736(17)30866-8.pdf