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1. Rheum RC > SSc/Mimickers/CTD-ILD > Flashcards

SSc/Mimickers/CTD-ILD Flashcards

1
Q

SSc classification

A

Localized: Morphea vs Linear scleroderma

Generalized: Limited vs Diffuse vs SSc sine scleroderma

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2
Q

Morphea manifestation

A

Single/multiple plaques on trunk SPARING hands/fingers

**NO ORGAN involvement

**Does NOT progress to SSc

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3
Q

Linear scleroderma manifestations

A

Bands of skin thickening on legs/arms, face

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4
Q

Limited scleroderma manifestation and AB

A

Calcinosis
Raynaud’s
Esophageal reflux
Skin thickening of neck, face, DISTAL to elbow/knees
Swollen fingers
Telangiectasia

MINIMAL ILD
PAH

AB:, ACA, PM-Scl, Th/To

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5
Q

Limited Scleroderma antibodies

A
  • Anticentromere
  • TH/TO
  • U1 RNP
  • PM-Scl
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6
Q

Diffuse Scleroderma antibodies

A
  • SCL70 (aka Anti-topoisomerase)
  • U3RNP
  • RNApol3
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7
Q

Associated diseases w/ this antibody: Anticentromere

A

Limited
PAH
Bad gut disease
PBC

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8
Q

Associated diseases w/ this antibody: TH/TO

A

Limited
PAH
ILD

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9
Q

Associated diseases w/ this antibody: U1-RNP

A

Limited
ILD
PAH

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10
Q

Associated diseases w/ this antibody: U3RNP

A

Diffuse
ILD
PAH
Myositis
SRC

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11
Q

Associated diseases w/ this antibody: PM-Scl

A

Limited
ILD
Myositis

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12
Q

Associated diseases w/ this antibody: SCL70

A

Diffuse
ILD
SRC

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13
Q

Associated diseases w/ this antibody: Anti-topoisomerase

A

Diffuse SSc
ILD
SRC

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14
Q

Associated diseases w/ this antibody: RNApol3

A

Diffuse SSC
SRC
Malignancy

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15
Q

Diffuse scleroderma manifestations

A

Skin thickening PROXIMAL to elbows/knees with truncal involvement
Friction rub
Raynaud’s, Puffy hands,
Arthritis, Carpal Tunnel
Severe ILD
PAH/PH
GI involvement

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16
Q

SSc mortality cause

A

Lung: ILD, PAH
Cardiac: HF, arrhythmia
Renal: SRC
Infection
Cancer

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17
Q

SSc sine scleroderma manifestations and AB

A

Internal manifestations of SSc
ANA (nuclear pattern), ACA, SCl70, RNApol
NO SKIN THICKENING

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18
Q

SSc Pathogenesis

A

Vascular damage (infxn, ROS, silica, etc) activates DCs via TLRs → Type 1 IFN →
- Th2 activation→ profibrotic IL4 and 13 production
- Activate M2 macrophages → TGFb → endothelial cell differentiation into mesenchymal cells
- B cell activation → IL6 and autoantibody production

Plt activation

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19
Q

SSc skin biopsy changes

A

Tissue fibrosis - excessive collagen fiber accumulation

Perivascular inflammation (T cells and monocytes)

Loss of hair follicles and sweat glands, and dermal capillaries (assoc’d w/ hypoxia → VEGF production)

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20
Q

Skin Tx Thickening SSc

A

MMF, MTX,
CYC, RTX
Toci
Abatacept
IVIG 2g/kg / month
HSCT

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21
Q

Skin Pruritus Tx SSc

A

Low dose pred (<15mg/d)
BZD
Topical: emolient, oatmeal bath
Gabapentin, pregabalin
Low dose naltrexone

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22
Q

Clues for early SSc in pt w/ Raynaud

A

Positive AB (nucleolar ANA, SCL70, ACA, P-SCl, RNP3)
Nail changes: capillary dropout, dilatation
Disease activity: Tendon friction rub, Ulcer, Infarct
Puffy hands, edema
Esophageal hypomotility (dilated esophagus)

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23
Q

** Patterns of nailfold capillaroscopy **

A

Early: few giant capillaries & capillary microhemorrhages. NO loss of capillaries. Preserved capillary distribution

Active: freq giant capillaries & capillary microhemorrhages. Mod loss of capillaries. Mild disorg of capillary architecture

Late: nearly absent giant capillaries & microhemorrhages. . Severe loss of capillaries. Capillary ramification 2/2 neoangiogenesis and disoganized capillaries

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24
Q

Raynaud Tx

A

Core/hand warming
STOP smoking

CCB, ARB, SSRI (fluoxetine)

Topical nitrates, PDE5i, Prostacyclin analogs, Endothelin ANT

Antiplatelet
Statins

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25
Q

Digital Ulcer NonPharm Tx

A

Avoid cold (Warming core, warming hands/feet),
Avoid trauma,
Smoking cessation,
Local wound care,
Avoid sympathomimetics

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26
Q

Digital Ulcer Vasodilator Tx
Which prevent new and which heal ulcer

A

Vasodilator:
HEAL ULCER and PREVENT new:
- CCBs = 1st line
- PDE5 inhibitors = 2nd line
- IV prostacyclin analogues = 3rd line

ONLY prevent new:
- Bosentan

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27
Q

Digital Ulcer Non-Vasodilator Tx

A

Statin,
Botox injections,
Analgesia, Digital sympathectomy,
Surgical debridement/amputation
Fat grafting,
Antibiotics if infection

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28
Q

Drugs to avoid in patients with ulcers

A

Vasoconstrictors: decongestants, Triptans

Stimulants: Amphetamines, Cocaine, ADHD medications

Weight loss,

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29
Q

Baseline testing SSc

A

HRCT
PFT (lung vol, spirometry, DLCO)
TTE
6MWT
EKG

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30
Q

Most common ILD in SSc

A

2/3 NSIP
1/3 UIP

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31
Q

Highest risk for progressive ILD

A

Older black/native man
Diffuse
SCL70 or Isolated Nucleolar ANA (ie NEG antiScl70)

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32
Q

Definition of ILD progression

A

Predicted FVC decline >10%
Predicted DLCO decline >15%

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33
Q

When to biopsy lung in SSc-ILD

A

Atypical lung injury on CT
Concern for alternative dx

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34
Q

** SSc ILD Tx**

A

Preferred: MMF, Toci, Ritux
Additional: CYC,AZA, Nintedanib

Others:
CNI (cyclosporine, tacro)
HSCT

Strong recommendation to avoid GC

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35
Q

When to consider HSCT

A

Early diffuse (<5y)
Mild/mod organ
Refractory/worsened on conventional immunosuppression

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36
Q

Tests before HSCT

A

PFT
HRCT
TTE
cMRI
RHC

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37
Q

Exclusion for HSCT

A

Smokers
Cardiopulmonary involvement
Severe internal organ

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38
Q

pHTN groups & which type in SSC

A

1: PAH
2: PH assoc’d w/ L heart dz
3: assoc’d w/ chronic hypoxia (eg ILD)
4: chronic thromboembolic-assoc’d PH
5: unclear/multifactorial (eg sarcoid)

1,2, 3 in SSC

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39
Q

** Right heart cath results in each type pHTN**

A

1:
Mean pulm artery pressure >20mmHg
Pulm capillary wedge pressure (PCWP) <15mmHg
Pulmonary vascular resistance >3woods (nonspecific for etiology)

2: Mean pulm artery pressure >25mmHg PCWP > 15mmHg

  1. Elevated pulm pressures due to chronic hypoxia
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40
Q

PAH manifestations

A

Dyspnea
Fatigue
Right heart dysfunction: TR, JVD, RV heave
Hepatomegaly and LE edema

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41
Q

** PAH risk factors in SSc**

A

Limited (esp longstanding)

AB: ACA, Isolated nucleolar pattern ANA

Sx: Longer duration Raynauds, Extensive telangiectasia

DLCO <60% w/o ILD or other cause
FVC/DLCO >1.6 (disproportionately low DLCO)

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42
Q

TTE findings of PH

A

Dilated RA & RV
RV dysfunction
Interventricular septal flattening
Pericardial effusion
Elevated RVSP (>40)

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43
Q

** PAH Tx**

A

CCB
Diuretics, fluid restriction, low salt
Warfarin for idiopathic, not SSc-assoc’d

**Prostacyclins (ie vasodilators) **(inhaled ilioprost, PO treprostinil, SC/IV epoprostenol, treprostinil)

**Endothelin R ANT (bosentan, ambrisentan, macitentan) **

Nitric oxide potentiators:
- PDEi (more cGMP for vasodilation) eg sildenafil or tadalafil;
- Guanylate cyclase stimulator eg riociguat

44
Q

Cardiac manifestations SSc

A

Cardiomyopathy (infiltrative/fibrotic on cMRI) - risk factors = blacks, diffuse skin, RNAPol3
Pericardial effusion (asymptomatic)
Pericarditis
CAD

45
Q

** SRC risk factors **

A

Sx: Early diffuse skin (1st 1-4years),
Friction rub

AB: RNAPol3

Tx: Steroids (pred >20 or prolonged low dose)
Cyclosporine

Proteinuria
HTN
CKD
Elevated ESR
Thrombocytopenia

46
Q

SRC manifestations

A

Arterial HTN >150/90 (10% normotensive)
Grade 3 retinopathy (flame shaped hemorrahge and/or cotton wool exudates) or Grade 4 (papilledema)
Rapid renal failure (within a month)
Pericardial effusion

47
Q

SRC labs

A

AKI
Proteinuria
Consumptive thrombocytopenia
Microangiopathic hemolysis (schistocytes)
Hemolysis labs: hapto, bili, LDH, DAT, smear,
Elevated renin
Normal/mildly decreased ADAMTS13

48
Q

ANCA GN vs SRC

A

ANCA GN:
No hemolytic manifestations
HTN does not predominate
RBC casts present
ANCA present
Biopsy shows AAV

49
Q

** SRC Tx and dose **

A

Short acting ACE (captopril, enalapril) → decrease sBP by 20 w/i 1st 24h
If still HTN: CCB, then AB

Captopril 6.25–12.5 mg every 8h, and increased to 50 mg TID

  • NO prophylaxis w/ ACE (worse prognosis bc can hide HTN)
50
Q

SRC poor prognostic markers

A

Male
Initial creatinine >3mg/dL
Late detection bc normal BP at onset
Cardiac involvement (myocarditis, arrythymia

51
Q

GI manifestation SSc

A

Upper GI:
- Dysphagia
- GERD
- Strictures
- Barrett’s
- GAVE

Lower GI:
- SIBO (constipation alternating diarrhea),
- Malabsorption
- Pseudoobstruction,
- Fecal incontinence (rectal spincter tone loss),
- Rectal prolapse

52
Q

SSc GI pathophysiology

A

Neural dysfcn from arteriolar changes of vasa nervorum → dysmotility

Smooth muscle fibrosis and atrophy

53
Q

How to assess upper GI manifestations

A

Manometry
Barium esophagram
Barium swallow (NOT if poor motility bc risk o impaction)
CT scan showing dilated esophagus
Endoscopy (relux esophagitis, candidiasis, Barrett’s, strictures)

54
Q

Esophageal dysmotility Tx SSc

A

Elevate head of bed
No food 2-3h pre bedtime
Antacids, H2 blockers, PPI
Motility agents:
- Metoclopramide, erythromycin → tachyphylaxis and GI smooth muscle fibrosis
- Domperidone and erythromycin → prolong QTc

Refractory: injectable octreotide or botox

55
Q

GAVE: What is it and how to tx

A

Gastric antral vascular ectasia (GAVE)

Telagiectasis of gastric mucosa → bleed/anemia

Tx: Laser or Argon plasma coagulation

56
Q

Small intestine manifestations SSc

A

Dysmotility →
SIBO →
Diarrhea →
Electrolyte abN and Malabsorption (low albumin, vit B6, B12, vit D folic acid) D-xylose absorption, beta carotene; high fecal fat)

57
Q

Large intestine manifestations SSc

A

Fecal incontinence
Wide mouth diverticulae (wall thinning/atrophy)
Rectal prolapse

57
Q

Dysmotility Tx SSc

A

Domperidone,
Metoclopramide,
Erythromycin,
Injectable ocreotide,
Fiber

58
Q

Diarrhea Tx SSc

A

Empiric tx for SIBO (rixamin 550mg TID, cipro 500 BID, amox clav 875 BID, or flagyll 500 TID x 10d)

AVOID drugs that slow motility (loperamide, paregoric) and FODMAP (fermentable oligosacch, disachh, monosacch, polyols)

Supplements if malabsorption

59
Q

Rectal involvement Tx SSc

A

Fecal incontinence → bioeedback, sacral nerve stim +/- surgery
Rectal prolapse: manage constipation +/- sugery

60
Q

What/Where calcinosis

A

Calcium phosphate
Hands (PIP, finger tips), periarticular, bony prominence (extensor elbow/knee)

61
Q

What diseases hv calcinosis

A
  • Idiopathic
  • Rheum: SSc, DM, SLE, Sarcoid, EDS
  • HyperCa: Paraneoplastic, HyperPTH, Milk Alkali, CKD, Calciphylaxis, Pagets
  • Infxn
  • Tumors
  • Pseudoxanthoma elasticum
  • Panniculitis
  • Trauma
62
Q

Calcinosis Tx

A

Weak evidence: steroids, Colchicine, Probenecid, Diltiazem

Topical/IV sodium thiosulfate BINDS Ca
**check BMD bc can bind any salt

IV bisphosphonate block OC and lower systemic Ca (if JDM)

Surgery (recurrence common)

63
Q

Telangiectasia - what and where

A

Dilated venules, capillaries, arterioles

Hands, face, lips oral mucosa

64
Q

Tx Telangiectasia

A

Laser therapy

65
Q

MSK involvement SSc

A

Acrosclerosis w/ osteolysis
Resorption (ribs, mandible, acromion, radius, ulnar)
Tendon friction rub (wrists, ankles, knees)

66
Q

Friction rub pathophys

A

Inflammation, edema, and fibrosis of tendon/tenosynovial sheath
- Increased fibrin deposition
- Increased thickness of tendon retinacula

67
Q

Muscle abnormalities in SSc, histology, and tx

A

Noninflammatory benign myopathy from inactivity/steroid use
- H\istology normal (type 2 muscle fiber atrophy), normal CK

Mild elevated CK w/ intermittent sx
Biopsy: interstitial fibrosis and fiber atrophy. Minimal inflammatory cell infiltration
NO steroids

Inflammatory myopathy (ie overlap/MCTD) - elevated CK. Tx w/ immunosuppression

68
Q

** Features that separate mimickers from SSc **

A

NO Raynaud’s
NO nailfold capillary abnorm (dilatation/ dropout)
NO ANA+
NO cutaneous sclerosis progression in centripetal fashion (finger/toe spreading proximally)
NO organ involvement (telangeictasia, ILD)
May see eosinophilia (eg in EF)

69
Q

** Mimickers of SSc **

A

Localized scleroderma (morphea, linear scleroderma)

Immune mediated:
- Eosinophilic fasciitis
- chronic GVHD

Deposition
- NSF (nephrogenic systemic fibrosis)
- Scleredema
- Scleromyxedema
- Amyloid

Metabolic (DM cheiroarthropathy, hypothyroid, phenylketonuria)
Paraneoplastic

Meds (Docetaxel, radiation, carbidopa, bleomycin, tryptophan, rapeseed oil)
Silica, pesticide, organic solvent, epoxy
POEM (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, skin changes)

70
Q

Morphea subtypes & characteristics

A

Circumscribed: most common in adults

Linear: MC children, unilateral limb or coup de sabre (scalp/face → neurologic sx or uveitis). Can → tissue atrophy

Generalized: 4+ (>3cm) involving 2+ body sites.
-Ulcers/necrosis, muscle atrophy, contracture.
-RISK cutaneous SCC.

Deep: rarest. Includes layers of fat, fascia, muscle. Symmetric, over extremities

71
Q

Morphea Tx

A

Acute:
- Topical (steroid, CNI, calcipotriene),
- Intralesional steroid
- Phototherapy
- PO prednisone,
- MTX, MMF, HCQ, IFX, cyclosporine

Chronic:
- PT/OT: for contracture/functional impairment
- Surgery: filler injection, autologous fat transfer

72
Q

Scleredema manifestions

A

Nonpitting woody skin edema
Neck, UPPER BACK, shoulder trunks, face, extremities
SPARES FEET/HANDS

Dysphagia, myalgia, contractures, diploplia

73
Q

Scleredema biopsy

A

Normal epidermis
Thickened dermis with mucin between collagen bands

74
Q

Scleredema Subtypes

A

1: AFTER infection or febrile illness, can cause carditis, myositis, CHF

2: Assoc’d w/ paraproteinemia (IgG, IgA), MM, amyloid, can precede Ca

3: Associated with diabetes

75
Q

Scleromyxedema manifestations

A

Wide spread small (2-3mm), nonpruritic, waxy papules
- Face, neck, upper trunk, distal forearms, dorsal hands - NO PALMS

Extracutaneous:
-Myopathy, Dysphagia,
-Arthritis,
-Dermato-neuro syndrome (fever, sz, coma s/p flu prodrome),
-Cardiac, pulm, renal
- Monoclonal gammopathy

76
Q

Scleromyxedema biopsy

A
  • Mucin deposition in dermis (normal epidermis)
  • Fibrosis: Fibroblast proliferation
  • Perivascular mononuclear cell infiltrate
77
Q

Scleromyxedema Tx

A

IVIG
Corticosteroid,
Thalidomide
MM tx (melphalan, CYC)

78
Q

EF stages

A

1: pitting edema of symmetric arms/legs (calves & forearms), ** SPARES HANDS/FEET **, NO finger/toes,

2: woody, peau d’orange, groove sign (indentated from dermis tethering to fascia & muscle along veins).
Induration of extremities, trunk, face.
NORMAL CK with low grade myositis

  1. Fascial involvement → early carpal tunnel
    Flexion contracture, muscle atrophy.

**NO sclerodactyly or nailfold change

79
Q

EF labs/imaging

A

Eosinophilia (20% normal; only in early stage; resolves w/ steroids)
ESR, CRP

Immune mediated aplastic anemia,
Thombocytopenia,

MDS, MM, lymphoma
Hypergammaglobulinemia
Aldolase elevated (normal CK)

MRI: fascial thickening and enhancement Gad

80
Q

EF biopsy instruction and result

A

** Full thickness deep wedge biopsy (for skin, subcutis, fascia, muscle) **

Inflamm infiltrate (lymph, plasma cells, histiocyte)

Eosinophilic infiltration in early disease

81
Q

EF complication if untreated

A

Joint contracture
Peau d’orange (bound down indurated skin)

Some self limited

82
Q

Poor prognostic features EF

A

Young age
Trunk involvement
Peau d’orange

83
Q

** EF Tx **

A

High dose pred (40-60mg) EARLY
Refractory: MTX > MMF
Case report: Toci, TNFi, Tofa, Ritux, IVIG
PT for flexion contracture

84
Q

Nephrogenic systemic fibrosis manifesetations

A

HISTORY OF GADOLINIUM EXPOSURE in CKD
ABSENCE OF MONOCLONAL GAMMOPATHY
NOT REVERSIBLE

Woody induration
Plaques
Cobble stone, Peau d’orange
Pruritus
Hyperpigmentation

Fibrosis of visceral organ : renal failure, cardiopulm, GI, diaphragm atrophy

NO Raynauds
NO FACIAL INVOLVEMENT

85
Q

NSF (nephrogenic systemic fibrosis) labs

A

No diagnostic labs.
NO paraprotein and scleroderma assoc’d AB
Normal SPEP

86
Q

NSF (nephrogenic systemic fibrosis) biopsy

A

NO inflamm cells
Increased dermal fibroblasts, collagen, mucin deposition

CD34+ fibrocyte and CD68+ monocytes

*similar to scleromyxedema but NO monoclonal gammopathy and NO face involvement in NSF

87
Q

NSF (nephrogenic systemic fibrosis) Tx

A

PREVENTION - no gad if stage 4/5 CKD
No treatment
Anecdotal: phototherapy, extracorporeal photophresis, PLEx, imatinib

88
Q

cGVHD manifestations

A

Trunk + extremities (vs SSc starts at digits)
Antibodies: sometimes Scl70, PM Scl, ANCA

**unique = underwent allogenic bone marrow transplant

89
Q

cGVHD prevention & Tx

A

Prevent:
- Pre-transplant antithymocyte globulin
- Post-transplant cyclosporine, IVIG, Ritux

Tx: Pred, Cyclosporine
Anecdotal: phototherapy, extracorporeal photopheresis, sirolimus, imatinib, thalidomide, Ritux

90
Q

Myositis ILD Tx

A

1st line: MMF, Aza, Ritux, CNI
Additional: JAKi, CYC

+Short term GC

91
Q

MCTD ILD Tx

A

1st line: MMF, Aza, Ritux
Additional: Toci, CYC

+Short term GC

92
Q

RA ILD Tx

A

1st line: MMF, Aza, Ritux
Additional: CYC

+Short term GC

93
Q

Sjogrens ILD Tx

A

1st line: MMF, Aza, Ritux
Additional: CYC

+Short term GC

94
Q

Autoimmune conditions associated with EF

A

PBC
SLE
Sjogrens

Immune mediated plastic anemia
Thombocytopenia

MDS, Lymphoma, Leukemia

95
Q

** 2 mimics of Scleroderma associated with cancer **

A

Scleredema type II,
Scleromyxedema,
Eosinophilic fasciitis

96
Q

** 2 REVERSIBLE mimics of Scleroderma **

A

Morphea,
Scleredema type 1,
Eosinophilic fasciitis (in some patient’s it is self-limited with spontaneous improvement, occasionally complete remission can occur).

97
Q

** EF trigger

A

Strenuous physical activity (eg marathon)

98
Q

3 cytokines important in scleroderma pathogenesis

A

IL 4
IL 13,
TGF beta

IL6, 8, 17
IFNg
TNF

99
Q

** 5 hand findings suggestive of scleroderm**

A
  • Arthralgias and inflammatory arthritis
  • Joint contractures
  • Tenosynovitis and tendon friction rubs
  • Skin sclerosis
  • Calcinosis (more so with limited disease)
  • Raynaud’s phenomenon and digital ulcerations
  • Puffy hands
  • Acro-osteolysis / Pulp loss
  • Nailfold capillary abnormalities
100
Q

Nailfold capillary findings in SSc

A
  • Enlarged, giant, dilated capillaries
  • Micro-hemorrhages
  • Capillary dropout
  • Neovascularization
  • Disorganization of capillary architecture
101
Q

What rheum diseases cause NSIP

A

Scleroderma
PM/DM
Sjogrens
MCTD
RA
AAV

102
Q

What rheum diseases cause UIP

A

RA
MPA
Scleroderma

103
Q

What rheum diseases cause COP

A

DM
RA
Sarcoid
SLE

104
Q

What rheum diseases cause LIP

A

Sjogrens
RA
SLE

105
Q

SRC biopsy pathology

A
  • Absence of inflammatory infiltrates
  • Fibrinoid necrosis
  • Arteriole THROMBOSIS → Glomerular ischemia
  • ONION SKIN LESIONS
  • Fibrointimal sclerosis
106
Q

** Causes of soft tissue calcification?**

A
  • Hyperparathyroidism
  • SSc
  • DM/PM/JDM
  • SLE, MCTD
  • Pseudoxanthoma elasticum (mineralization of elastic fibers)
  • Panniculitis
  • Trauma – heterotropic ossification and injection granulomas
  • Gout/CPPD
  • CKD - metastatic calcification
  • Tumoral calcinosis (FGF23 mutation)

1. Rheum RC (23 decks)

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