Systemic Sclerosis

Question 1

Mechanism

Answer 1

Autoimmune microangiopathy causes fibroblast activation and collagen deposition.

Question 2

Fibrosis driver 1

Answer 2

TGF-β is a key profibrotic cytokine in systemic sclerosis.

Question 3

Fibrosis driver 2

Answer 3

Endothelin-1 promotes vasoconstriction and fibrosis in systemic sclerosis.

Question 4

Diffuse SSc antibody

Answer 4

Anti-Scl-70 (topoisomerase I).

Question 5

Diffuse SSc features

Answer 5

Interstitial lung disease, renal crisis, cardiac fibrosis.

Question 6

Limited SSc antibody

Answer 6

Anti-centromere antibody.

Question 7

Limited SSc features

Answer 7

Pulmonary hypertension and CREST syndrome.

Question 8

Sine SSc definition

Answer 8

Systemic sclerosis without skin involvement.

Question 9

Overlap SSc antibodies

Answer 9

Anti-PM-Scl or Anti-U1-RNP.

Question 10

CREST C

Answer 10

Calcinosis.

Question 11

CREST R

Answer 11

Raynaud phenomenon.

Question 12

CREST E

Answer 12

Esophageal dysmotility.

Question 13

CREST S

Answer 13

Sclerodactyly.

Question 14

CREST T

Answer 14

Telangiectasia.

Question 15

NSIP pattern

Answer 15

Ground-glass opacities; better prognosis.

Question 16

UIP pattern

Answer 16

Honeycombing; fibrotic and worse prognosis.

Question 17

Vascular feature 1

Answer 17

Raynaud phenomenon.

Question 18

Vascular feature 2

Answer 18

Telangiectasia.

Question 19

Vascular feature 3

Answer 19

Digital ulcers.

Question 20

Renal crisis clue

Answer 20

Sudden severe hypertension and acute kidney injury.

Question 21

Renal crisis treatment

Answer 21

Immediate ACE inhibitor (captopril).

Question 22

GI feature 1

Answer 22

Gastroesophageal reflux.

Question 23

GI feature 2

Answer 23

Small intestinal bacterial overgrowth.

Question 24

GI feature 3

Answer 24

Malabsorption.

Question 25

Cardiac feature 1

Answer 25

Conduction abnormalities.

Question 26

Cardiac feature 2

Answer 26

Myocarditis.

Question 27

Cardiac feature 3

Answer 27

Restrictive cardiomyopathy.

Question 28

Antibody PAH

Answer 28

Anti-centromere antibody.

Question 29

Antibody ILD

Answer 29

Anti-Scl-70 antibody.

Question 30

Antibody renal

Answer 30

Anti–RNA polymerase III antibody.

Question 31

Screening frequency

Answer 31

Annual echocardiogram and BNP measurement.

Question 32

PAH screening tool

Answer 32

DETECT algorithm for SSc >3 years and DLCO <60%.

Question 33

Raynaud first-line

Answer 33

Nifedipine (calcium-channel blocker).

Question 34

Raynaud second-line

Answer 34

Sildenafil (PDE5 inhibitor).

Question 35

Raynaud third-line

Answer 35

IV iloprost (prostacyclin analogue).

Question 36

Raynaud fourth-line

Answer 36

Bosentan (prevents new digital ulcers).

Question 37

ILD first-line

Answer 37

Mycophenolate mofetil (MMF).

Question 38

ILD second-line

Answer 38

Cyclophosphamide (CYC).

Question 39

ILD add-on

Answer 39

Nintedanib slows FVC decline by ~52 mL/year (SENSCIS NEJM 2019).

Question 40

ILD inflammatory option

Answer 40

Tocilizumab preserves FVC (focuSSced 2020).

Question 41

ILD refractory option

Answer 41

Rituximab (RECITAL 2023).

Question 42

PAH therapy

Answer 42

Endothelin antagonist + PDE5 inhibitor ± prostacyclin.

Question 43

Renal crisis prevention

Answer 43

Avoid prophylactic ACE inhibitors and high-dose steroids.

Question 44

Rapid dcSSc treatment

Answer 44

Autologous HSCT (ASTIS JAMA 2014, SCOT NEJM 2018).

Question 45

HSCT mortality risk

Answer 45

Treatment-related mortality approximately 7–10%.

Question 46

Exam trap 1

Answer 46

Steroids >15 mg can trigger renal crisis.

Question 47

Exam trap 2

Answer 47

ACE inhibitors are started only during renal crisis.

Question 48

Exam trap 3

Answer 48

Low DLCO with normal lung volumes indicates pulmonary hypertension.

Question 49

Prognosis statistic

Answer 49

Five-year survival is approximately 80%.

Question 50

Prognosis cause of death

Answer 50

Interstitial lung disease is the leading cause of death.

Question 51

EBM trial MMF

Answer 51

SLS II (2016) showed MMF ≈ CYC for ILD with fewer adverse events.

Question 52

EBM trial Nintedanib

Answer 52

SENSCIS (2019) showed nintedanib slowed FVC decline.

Question 53

EBM trial Tocilizumab

Answer 53

focuSSced (2020) showed tocilizumab preserved FVC in early dcSSc.

Question 54

EBM trial Rituximab

Answer 54

RECITAL (2023) showed rituximab ≈ CYC with fewer adverse events.

Question 55

EBM trial HSCT

Answer 55

ASTIS (2014) and SCOT (2018) showed HSCT improved survival but had TRM risk.

Question 56

EBM screening

Answer 56

DETECT (2013) validated annual PAH screening in SSc.