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MyDermatology-Bolognia > Ch. 44-Sjogrens, Still's, Relapsing polychondritis > Flashcards

Ch. 44-Sjogrens, Still's, Relapsing polychondritis Flashcards

1
Q

What features are required for a diagnosis of systemic onset JIA

A
  1. High episodic fevers daily for 2 weeks
  2. Atleast one of: evanescent rash, splenomegaly, hepatomegaly, serositis, general LAD
  3. Symmetric poly arthritis > oligo arthritis (erosive in 20%) –> onset may be delayed months to years
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2
Q

Name 6 lab abnormalities in juvenile stills

A 
Elevated ferritin
Elevated ESR/CRP
Anemia
Leukocytosis with neutrophilic
Thrombocytosis 
*RF and ANA neg
Polyclonal gammopathy
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3
Q

What type of immune system activation is seen in juvenile Still’s

A

Innate immune with Il-1, TNF alpha and IL-6 production

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4
Q

Name 6 signs and symptoms Still’s

A
  1. Daily spiking fevers >38.9 often in evening
  2. Arthralgia, often coincide with fevers
  3. Arthritis-often polyarticular and symmetric, can come at same time rash onset or months to years later, often knees, ankles, hips, small hand joints
  4. Transient evanescent rash
  5. lymphadenopathy
  6. hepatosmplemegaly
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5
Q

Describe 4 cutaneous characteristics of juvenile stills

A
    • transient, evanescent pink salmon coloured rash often coincides with fevers
    • predilection to axillae, waist, linear lesions/flagellate erythema
  2. periorbital edema/erythema
  3. may get RA nodules
  4. persistent plaques, which also may be linear
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6
Q

Name 2 path findings show of juvenile Stills

A

-perivascular and interstitial neutrophil-dominant mixed infiltrate –> “neutrophilic urticarial dermatosis”

A variable number of dyskeratotic keratinocytes, located primarily in the upper epidermal layers, is also a typical finding.

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7
Q

what % sJIA have persistent arthritis

A

In ~40–50% of patients, the arthritis resolves completely.

50% of the children may have a chronic course that includes persistent arthritis

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8
Q

Name 6 treatment options sJIA

A 
NSAIDS for mild articular disease 
Steroids +- MTX
Leflunomide
Aza
Abatacept
Anti-tnf (less for systemic JIA >JIA)
Anti-IL-1 (anakinra)
Anti IL-6 (toci)
Stem cell transplant
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9
Q

Average age onset AOSD?

A

Before age 30 in majority, usually young adults

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10
Q

What type of immune response is seen in stills, name 5 cytokines elevated?

A

Th1 response

TNFalpha
IFN gamma
IL-2
IL-6 
IL-18
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11
Q

Name 6 non skin manifestations AOSD

A
  1. Fever-often 39+, daily in evening
  2. Sore throat
  3. Constitutional sx
  4. Hepatomegaly > splenomegaly
  5. Arthritis- symetric, knees wrists ankles, present in 65-100%, joint pain often with fever spike,
  6. carpal ankylosis-minimal pain but difficulty moving carpal bones, can also occur DIP, PIP and C-spein but sparing MCPS
  7. Other organists can occur but rare (pull fibrosis, pleuritic, pericarditis, myocarditis, nephritis
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12
Q

Name 5 lab findings in AOSD

A 
elevated ESR/CRP
Elevated ferritin 
thrombocytosis
Leukocytosis
Anemia
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13
Q

Describe the skin rash in AOSD

A 
Salmon-pink
Asymptomatic 
Transient, occurs with fever spikes
Macular
Points of pressure--> most often on trunk but can be extremities 
Koebner and flagellate erythema (rare)
Eyelid edema (rare)

Violaceous to reddish brown, scaly, persistent papules and plaques can also occur

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14
Q

Pathology findings AOSD

A

Same as sJIA

mixed perivascular and interstitial infiltrate composed of neutrophils and lymphocytes–> “neutrophilic urticarial dermatosis”

dyskeratotic keratinocytes

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15
Q

Manistay treatment of stills? Name 4 other options

A 
Prednisone first line
MTX second line
Toci (Îl-1)  or anti IL-6
Ritux
Anti-tnf
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16
Q

Relapsing polychondritis: For a diagnosis you need bx proven chondrites in 2/3 sites. What 3 sites?

A

Auricular
Nasal
Laryngotracheal

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17
Q

If only 1 bx site proven chondrites, you need what other criteria to make diagnosis?

A 
At least 2 of the following:
ocular inflammation
vestibular dysfunction
hearing loss
 seronegative inflammatory arthritis
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18
Q

Average age onset polychondritis?

A

20-60

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19
Q

Race most commonly affected polychondritis?

A

Caucasian

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20
Q

Proposed antigen being attacked in relapsing polychondritis?

A

Type 2 collagen

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21
Q

What % patients with relapsing polychondritis have autoantibodies ?

A

50% against type II collagen

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22
Q

Describe the cutaneous clinical features auricular chondrites

A

Erythema, swelling and pain of cartilaginous portion auricle, can last days to weeks, compromise hearing, eventually develop scarring and destruction of cartilage, sparing the earlobe

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23
Q

What are the most common sites of chondritis

A

Ear/auricle
Nose
Costochondral joints
Larynx, trachea, bronchi (respiratory tract)

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24
Q

Describe 10 manifestations of relapsing polyhchondritis

A
  1. Auricular chondritis
  2. Nasal chondritis and saddle nose deformity (pain, stuffiness, crusting, rhinorrhea, epistaxis and compromise of olfaction can occur)
  3. Chostochondral chondritis
  4. Respiratory tract chondritis with cough, hoarseness, choking, dyspnea, wheezing, or tenderness to palpation of the anterior neck in sites overlying the larynx or trachea –> airway obstruction or collapse, flail chest, and secondary pulmonary infections.
  5. Arthritis-asymetric, migrating, oligo
  6. Ocular inflammation
  7. Cutaneous lesions
  8. Renal- GN, AIN
  9. cardiovascular-aortitis, peri and myocarditis, conduction defects
  10. neurologic - cranial nerve palsies, vasculitis peripheral nerves
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25
Q

Name 7 non-cartilage cutaneous lesions in relapsing polychondritis

A 
Apthae
SVV
annular urticarial lesions
lived reticularis
sweets
EED
Erythema nodosum
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26
Q

What is MAGIC syndrome

A

Mouth and genital ulcers and inflamed cartilage

-relapsing polychondritis and beeches overlap

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27
Q

Name 2 diseases associated with relapsing polychondritis

A

Myelodysplastic syndrome

Other autoimmune disorders

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28
Q

What features increase risk MDS in relapsing polychondritis

A

Apthous ulcers

Small vessel vasculitis

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29
Q

Pathology of relapsing polychondritis?

A

Neutrophilic infiltrates early–> lymphoplasmacytic later–> eventual fibrosis and granulation tissue

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30
Q

Name 10 things on ddx for nasal deformity or destruction

A

Infectious–> Bacterial, leprosy, tb/lupus vulgarisms, rhinoscleroma, dimorphic fungi (parcocci), syphillis (tertiary), mycobacterial, aspergillosis, leish
Inflammatory–> GPA, sarcoid, SAVI, PLAID,
Neoplastic–> Nasal NK T cell lymphoma (anglocentric T cell lymphoma), SCC, BCC, sarcomas
Cocaine use
Factitious/trauma

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31
Q

Most common causes of death in relapsing polychondritis

A

pneumonia, systemic vasculitis, airway collapse and renal failure

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32
Q

List 6 treatments for relapsing polychondritis

A 
Prednisone mainstay
NSAIDs and colchicine
HCQ
Other immune suppresants--> MTX. AZA, cyclosporine, MMF, cyclophosphamide)
Anakinra
Toci
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33
Q

4 factors associated with poor prognosis in SJogrens

A

vasculitis
hypocomplementemia
cryoglobulinemia
parotid enlargement

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34
Q

What are the ACR criteria for Sjogrens?

A

Score 4+
Must meet inclusion criteria and exclusion criteria

  1. SSA/RO + = +3
  2. Labial salivary gland with focal lymphocytic sialadenitis and focus score of ≥ 1 foci/4 mm = +3
  3. Ocular staining score 5+ in one eye
  4. Schirmer test less than or equal to 5mm in one eye/5 min
  5. Whole salivary flow rate less or equal to 0.1 ml/min
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35
Q

What are the inclusion criteria for Sjogrens ACR criteria

A

Must be yes to at least one:

(1) Have you had daily, persistent, troublesome dry eyes for more than 3 months?
(2) Do you have a recurrent sensation of sand or gravel in the eyes? (3) Do you use tear substitutes more than 3 times a day?
(4) Have you had a daily feeling of dry mouth for more than 3 months?
(5) Do you frequently drink liquids to aid in swallowing dry food

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36
Q

What are 7 exclusion criteria of Sjogrens (can’t have these)

A
  1. AIDS
  2. Sarcoid
  3. Amyloid
  4. H/N radiation
  5. Active Hep C
  6. GVHD
  7. IgG4-related disease
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37
Q

What medications should be stopped before evaluating for SJogrens

A

Anticholingergics

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38
Q

Prevalence of Sjogrens in population? M:F ratio? Average age onset?

A

F:M 9:1

0.3-0.6% pop’n

4th/5th decade in onset

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39
Q

3 most common disease associations in Sjogrens (Secondary sjogrens)

A

rheumatoid arthritis
systemic lupus erythematosus (LE)
scleroderma

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40
Q

What is the disease with 20x increased risk in Sjogrens

A

Lymphoma

*These B-cell lymphomas are often extranodal and can originate in salivary as well as lacrimal glands

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41
Q

Major defining features of Sjogrens

A

Xeropthalmia–> can lead to e keratitis, corneal thinning and ulceration, and recurrent infections
Xerostomia–> can lead to dental caries, salivary gland infections, GERD, thrush,
Vaginal dryness–> candida

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42
Q

How to test for xeropthalmia

A

Sschirmer test
or
Corneal evaluation by optho–> fluorescein

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43
Q

How to test for xerostomia

A

Salivary gland scintigraphy
Sialometry
Parotid gland sialography
*Salivary gland biopsy most commonly done

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44
Q

Most common cutaneous manifestation Sjogrens

A

Xerosis with pruritus

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45
Q

Name 7 cutaneous manifestations Sjogrens other than xerosis

A 
*Purpura palpable and non palpable, petchiae--> lots of reasons
Cutaneous SVV
Hypergammaglobulinemic purpura of waldenstrom--> capillarities or petechiae
Urticarial vasculitis
Annular erythema
Erythema nodosum
Nodular amyloidosis
Sweets syndrome
Cryoglobulinemia
Raynauds
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46
Q

Name 3 RF for developing B cell lymphomas and increased mortality

A

vasculitis
cryoglobulinemia
hypocomplementemia

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47
Q

Extraglandular sjogrens organ involvement, name 5

A

Lungs-interstitial pneumonitis
Renal -interstital nephritis
Bone marrow
Neuro–> peripheral neuropathy, short term memory loss, depression, hearing loss
Arthritis*–> polyarticular, non-erosive, chronic and progressive, and it can be asymmetric, knees and ankles

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48
Q

What type of lymphoma are Sjogren’s patients at risk of developing

A

B-cell lymphomas, often extranodal in origin (e.g. salivary glands, lacrimal glands) and usually marginal zone lymphomas of the MALT origin

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49
Q

Name 5 lab findings in Sjogrens

A 
SSA- 60-80%
SSB-40-60
ESR
RF
Hypergammaglobulinemia
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50
Q

Treatment xerophthalmia

A
  • artificial tears
  • punctal plugs
  • topical cyclosporine
  • home humidifiers
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51
Q

Treatment xerostomia

A

Lifestyles: stop smoking, reduce salty/spicy/acidic foods and drinks, lower sugars/carbs (dental caries), avoid anti-histaminesand anticholinergics, use sodium bicarb rinses, humidifiers, DENTAL CARE

Saliva substituteS: Biotene spray or gel, lozenges

Saliva stimulants:

  • local–> xylitol containing gums/lozenges
  • systemics: Pilocarpine, cimevuline
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52
Q

When to consider systemic treatment in sjogrens

A

Cutaneous vasculitis or internal organ involvement

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53
Q

Name 4 systemic treatments for Sjogrens

A

Prednisone
MTX
MMF
AZA

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54
Q

What antibody associated with MTCD

A

U1RNP

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55
Q

Major cause of death in MCTD

A

pulmonary hypertension

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56
Q

MCTD encompasses which AI diseases?

A

SLE-less likely renal
Systemic sclerosis-often pHTN
Myositis/polymyositis (rare to see DM findings)
RA-arhtirits ranges from mild to erosive

57
Q

Name 6 core features of MCTD

A
  1. high-titer IgG anti-U1 ribonuclear protein (U1RNP) antibodies,
  2. Raynaud phenomenon-ulcers can occur
  3. swollen hands or sclerodactyly
  4. myositis-inflammatory, no DM usually
  5. esophageal dysmotility and GERD
  6. arthritis-mild to erosive
  7. pHTN in 25%
  8. Pleurisy and pericarditis
58
Q

Name 8 cutaneous findings in MCTD

A
  1. Raynauds with ice pick digital infarcts, often nail fold capillaries show dropout
  2. Scleredema and sclerodatyly
  3. Poikilodermatous lesions on upper trunk and extremity
  4. ACLE or SCLE lesions
  5. Livedoid vasculopathy
  6. cSVV
  7. Calcinosis cutis
  8. cutaneous mucinosis
  9. RA nodules
  10. Oral mucosal lesions–> ulcers, septal perforation
59
Q

2 lab findings suggestive pHTN in MCTD

A

APLS labs +

NtPROBNP

60
Q

3 labs tests to help distinguish MCTD from SLE

A

Lack of:

  • hypocomplementemia
  • anti-dsDNA
  • anti-Sm antibodie
61
Q

How to distinguish MCTD vs. SSc

A

Severity arthritis and myositis

62
Q

Name 10 cutaneous manifestations in or seen in RA

A

Palisading granulomas

  • RA nodules
  • Palisaded neutrophilic and granulomatous dermatitis (PNGD) and interstitial granulomous dermatitis (IGD)

Neutrophilic

  • Pyoderma gangrenosum
  • Sweets
  • Neutrophilic lobular panniculitis
  • rheumatoid neutrophilic dermatitis

Vascular

  • Bywaters lesions
  • small and medium vasculitis
  • feltys
  • EED
  • Intravascular/intralymphatic histiocytosis
  • Superficial ulcerating rheumatoid necrobiosis.
  • drug cutaneous manifestations related separately
63
Q

What % patients with RA get nodules

64
Q

What is a RF for rheumatoid nodules?

A

RF+ moderate to high titres

65
Q

Where do rheumatoid nodules occur? What do they look like

A

Extensor surfaces and pressures points
Periarticular

*Forearms extensor, around elbow, around MCPS, PIPs and DIPS

Firm/Semi-mobile papulonodules often asymptomatic, skin coloured

66
Q

What 2 medications are though maybe to stimulate nodulous?

A

MTX
TNF’s
*Also associated usually with steroid tapers tho so hard to know

67
Q

What drug can cause a papular eruption whose histologic features overlap with those of interstitial granuloma annulare and interstitial granulomatous dermatitis

68
Q

Pathology for RA nodules

A

Acute or early lesions may show leukocytoclastic vasculitis and/or an interstitial neutrophilic infiltrate.

central zone of eosinophilic fibrin surrounded by palisaded histiocytes, deep dermis or subcutis

69
Q

DDX RA nodules

A

Subcutaneous granuloma annulare
gouty tophi
Synovial hyperplasia/cysts (softer, painful)

70
Q

3 RF for RA vasculitis

A

high titre RF
severe erosive arthritis
RA nodules

71
Q

When does RA vasculitis typically appear

A

Late stage

72
Q

What % RA patients get vasculitis

A

2-5%, but up to 1/3 autopsies

73
Q

How does RA vasculitis present on the skin

A

with palpable and non-palpable purpura in small vessel disease

nodules, ulcerations, necrotizing livedo reticularis, and/or digital infarcts in medium-sized vessel disease

74
Q

Name 7 extra cutaneous manifestations RA vasculitis

A 
neuropathies
cerebral infarction
scleritis
alveolitis
carditis
 intestinal ulcers
 renal involvement with proteinuria.
75
Q

Most common non cutaneous manfiestiaon RA vasculitis

A

Neuropathy-mononeuritis multiplex

76
Q

If suspect RA vasculitis but can’t get cutaneous biopsy, next step?

A

Nerve conduction studies follower by sural nerve bx or muscle bx

77
Q

Mortality rate RA vasculitis

A

AS high as 40%

78
Q

What are by waters lesions

A

Purpuric papules distal digits like pulp or nail fold thromboses

Path shows LCV

Not associated systemic vascular

79
Q

What is Felty’s syndrome

A

Seropostiive RA
Splenomegaly
Neutropenia
Leg ulcers-often treatment resistant, pretibial

79
Q

What is Felty’s syndrome

A 
Seropostiive RA
Splenomegaly
Neutropenia-increase infix risk
Leg ulcers-often treatment resistant, pretibial
Increased risk lymphoma and leukaemia
80
Q

Causes felty syndrome ulcers

A 
Multifactorial, could be:
PG
Vasculitis
Ulcerative PNGD 
venous HTN
Neuropathy
81
Q

What does Rheumatoid neutrophilic dermatitis resemble?

A

Clincally/histopathologically resembles Sweet’s

Often in seropositive RA
-erythematous urticarial papules and plaques that are persistent and asymptomatic, but occasionally ulcerate. Lesions are symmetrically distributed, most commonly on the extensor forearms and hands, but they can occur elsewhere.

82
Q

How to tell PAN from RA vasculitis on path

A

Direct immunofluorescence in rheumatoid vasculitis shows prominent deposition of IgM as well as C3 in small and medium-sized vessels,

in polyarteritis nodosa the IgM and C3 vascular deposits are weaker and sparse and limited to medium-sized vessels.

83
Q

Main ddx based on lab markers for RA vasculitis

A

Cryoglobulinemia due to + RF, vasculitis and low complements

*Cryo typically low C4 tho, lower titre RF

84
Q

Ddx ulcers in RA

A 
PG
Vasculitis
Infection from immunosuppresion
Ulcerative PNGD
Secondary APLS
Angiocentric lymphomas 
Thromboembolic
Suprecifical ulcerating necrobiosis
85
Q

Ddx palisaded necrotizing granuloma

A 
EGPA
GPA
Rheumatic fever nodule
Palisaded neutrophilic granulamtous dermatitis - in association with AI-CTD (RA, vasculitis)
Rheumatoid nodule
86
Q

PAlisaded necrotizing granuloma with basophilic collagen degeneration, neutrophil dominant, C-ANCA +?

A

Most likely GPA

87
Q

Palisaded necrotizing granuloma with basophilic degeneration or eosinophilic degeneration, eos dominant?

88
Q

Palisaded necrotizing granuloma, basophilic collagen degeneration, netrophil dominant and P-ANCA + or ANCA neg?

A

AI-CTD like RA or vasculitis

89
Q

Palisaded necrotizing granuloma eosinohpilc degeneration that is neutrophil dominant? NExt step and ddx

A

RF+ —> Rheumtoid nodu;e

RF- —> Rheumatic fever nodule

90
Q

Name 2 treatments RA nodules

A

Excision-but often recurs

ILK- often not complete resolution

91
Q

Name 3 treatment options rheumatoid neutrophilic vasculitis

A

Steroids
DApsone
Colchicine

92
Q

Name 2 treatments Feltys

A

G-CSF and/or splenectomy

93
Q

Main tx for aggressvie RA vasculitis

A

Pulse IV pred + cyclo and plex

94
Q

Name 3 tx options SVV in RA

A

MMF or AZA
*MTX often doesnt work
TNF’s
Ritux

95
Q

Name 2 tx options RA PG

A

Steroids +Cyclosporine

TNFs

96
Q

What is PAPA syndrome? What is the gene mutation?

A

Pyogenic sterile arthritis
Pyoderma gangrenosum
Acne

Mutation in PSTPIP1
Protein: CD2 binding protein 1

97
Q

What is mode inheritance PAPA

98
Q

What is PASH?

A

Pyoderma gangrenosum, acne, suppurative hidradenitis

99
Q

What is PAPASH

A

Pyogenic arthritis, pyoderma gangrenosum, acne, supparative hidradentitis

100
Q

Name 6 hereditary periodic fever syndromes

A

Familial Mediterranean Fever (FMF)
TNF receptor associated periodic fever (TRAPS)
Hyperimmunoglobulinemia D with periodic fever syndrome (HIDS)

CAPS-Cryoporin associated periodic syndromes

  1. NOMID- Neonatal onset multisystem inflammatory disease
  2. Muckle Wells
  3. FCAS
101
Q

What is mode of inheritance FMF

102
Q

What ethnicity does FMF affect

A 
Turkish
Sephardic jewwish (spain and portugal)
Italian
Armenian
Arab
103
Q

What gene is mutated in FMF? What protein?

A

Gene: MEFV
Protein: Pyrin

104
Q

Average attack length in FMF

105
Q

What are the cutaneous findings of FMF

A

Erysipeloid erythema and edema
Favors lower leg and foot
LCV/IgA vasculitis in 5-10%

106
Q

Name 5 non cutaneous findings in FMF

A 
Peritonitis > pleuritis >pericarditis
Monoarthritis > exercised induced myalgia 
Rarely aseptic meningitis 
Acute scrotal swelling
Splenomegaly
107
Q

Name 1 laboratory finding in FMF

A

Low C5a inhibitor in serosal fluids

108
Q

Treatment FMF-Name 3

A
  1. Colchicine ppx
  2. NSAIDS
  3. TNF inhibitors
  4. Thalidomide
  5. Herbal remedies?
109
Q

Mode of inheritance HIDS

110
Q

Gene mutated in HIDS? Protein?

A

MVK
Protein: Mevalonate kinase
Key enzyme cholesterol pathway

111
Q

Lengthj of attack in HIDS

112
Q

Cutaneous lesions in HIDS

A

Erythematous macules and edematous papules which may become purpuric
Occasional vaginal and oral ulcers
Widepspread on face , trunk and extremities

Common to get HSP/LCV

113
Q

Average onset of HIDS

114
Q

Typical flare signs/symptoms HIDS

A 
Fever
Skin 
Cervical LAD
Hepatosplenomegaly 
Abdominal pain, rarely serositis
Arthralgias 
HEADACHE
115
Q

What lab value is elevated in HIDS

A

High serum IgD (>100)

High IgA1 mevalonate in urine

116
Q

Mode inheritance of HIDS

117
Q

Name 4 treatment options HIDS

A

Steroids acute attacks
Anakinra
TNF inhibitors
Simvastatin

118
Q

Mode inheritance of TRAPS

119
Q

Gene and protein mutated in TRAPS

A

Gene: TNFRSF1A

ProteinL TNF receptor 1A

120
Q

Length of attacks in TRAPS

A

Often over 1 week

121
Q

Cutaneous features of TRAPS

A

Erythematous patches and edematous plaques often with annular or serpiginous
LAter ecchymotic
Rarely oral ulcers
Migrate distally on an extremity with underlying myalgia, may be more widespread

122
Q

Flare symptoms of TRAPS

A

Skin
Peritonitis>Pleuritis and pericarditis
Migratory myalgia>Arthralgia >monoarthritis
Periorbital edema, conjuctivitis, rarely uveitis
Headache
Scrotal pain, splenomegaly, occasional LAN

123
Q

Name 2 tx options TRAPS

A

Steroids

TNF inhibitor

124
Q

Lab marker TRAPS

A

Low serum soluble TNF receptor-1 between attacks

125
Q

What gene is mutated in the cryoporin associated periodic syndromes

A

NLRP3

Protein: Cryoporin

126
Q

Mode inheritance of CAPS

A

AD for all of them

127
Q

What chromosome is NLRP3 on?

128
Q

What ethnic group typically sffected by CAPS

A

Any ethnic group

129
Q

How does MWS present?

  • flare length
  • cutaneous findings
  • extra cutaneous findings
A

1-2 days
Urticarial papules and plaques-widesapread on face, trunk, extremities
Non skin:
-abdo pain but rarely serositis
-Myalgias with lancinating limb pain
-arthralgias, sometimes large joint oligoarticular arthritis
-conjuctivitis, episcleritis, optic disc edema
-sensorienrural hearing loss
-headache

130
Q

What % MWS develop amyloidosis

131
Q

Name 4 tx options MWS

A

Steroids

IL-1 antagonists : Rilonacept, canakinumab, Anakinra

132
Q

Attack length in FCAS

A

Minutes-3 days

133
Q

Cutaneous findings in FCAS

A

Cold induced urticarial papules and plaques

Extremities >trunk and face

134
Q

Non cutaneous flare signs and symptoms in FCAS

A

Arthralgia>myalgias
Conjuctivitis
HEadache

135
Q

Treatments FCAS: NAme 1

A

IL-1/IL-1R antagonists

136
Q

Describe 8 features of NOMID

A
  1. Skin-urticarial papules and plauqes, occasionally ulcers on face, trunk extremities
  2. Often continuous disease, flares in between
  3. MSK: Epiphyseal and patellar overgrowth, arthritis, deforming arhtropathy
  4. Eyes: Conjuctivitis, uveitis, optic disc edema, blindness
  5. Neuro: sensorineural hearing loss, aseptic meningitis, seizures
  6. Lymph: LAN, HSM
  7. Dysmorphic facies, frontal bossing, protruding eyes
137
Q

Treatment NOMID

A

Il-1/IL-1R antagonists

MyDermatology-Bolognia (31 decks)

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