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Systemic Sclerosis + Sjogrens Flashcards

1
Q

What forms the CREST syndrome

A 
Calcinosis cutis
Raynaud's phenomenon
Esophageal dysmotility
Sclerodactyly
Telangiectasia

Note CREST = limited Scleroderma

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2
Q

What is sine scleroderma

A

Typical features of systemic sclerosis (usually features of limited subtype) without sclerosis or thickening of skin

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3
Q

What is the epidemiology of systemic sclerosis

A

More common in females 3:1
Peak age of onset 40-60
More common in African Americans (tends to be diffuse and more severe disease)

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4
Q

What are the 3 pathogenic features of systemic sclerosis

A
  1. Vascular injury
  2. Cellular and humeral autoimmunity
    - autoantibodies, T cells found in skin biopsies
  3. Progressive and vascular fibrosis
    - widespread tissue fibrosis with accumulation of type 1 collagen
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5
Q

What are 3 differentiating features of limited systemic sclerosis

A
  1. Slowly progressive skin disease limited to distal extremities, occasionally face
    - does not extend above elbows
  2. Long history of Raynaud’s prior to other manifestations
  3. Late pulmonary HTN, GI/malabsorptive issues
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6
Q

What are the 3 characteristic features of diffuse systemic sclerosis

A
  1. rapidly progressive skin disease starting in the fingers and ascending to involve face and trunk, proximal limbs (above elbows)
  2. Skin changes often start with an oedematous and itchy phase
  3. Other organ involvement within weeks - months
    - pulmonary fibrosis, renal disease, GI and myocardial involvement more common
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7
Q

What is CREST more common in

A

Limited systemic sclerosis = CREST syndrome

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8
Q

General features of systemic sclerosis

A

Fatigue, stiff joints, weakness, pain, sleep disturb, skin discolouration, erectile dysfunction, hypothyroidism, dry eyes and mouth, myopathies, neuropathies

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9
Q

What are triggers for Raynaud’s syndrome

A 
Cold
Change in temp
Vibration
Emotional stress
Drugs - b-lockers, cisplatin, bleomycin
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10
Q

What percentage of population have raynauds

A

5% of males, 10% females

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11
Q

What are the 3 phases of Raynaud’s

A

Vasoconstriction (white)
Ischaemia (blue)
Re-perfusion (red)

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12
Q

What are some skin changes with systemic sclerosis

A 
Initial odema and puritis
Discolouration and hypo pigmentation (salt and pepper)
Loss of hair follicles, sweat and sebaceous glands
Fixed flexion contractures
Microstomia
Pinched beak like nose
Expressionless face
Telangiectasia (face, hands, lips and oral mucosa)
Calcinosis cutis
Tendon friction rubs
Nail fold capillary dilatation
Sclerodacytly
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13
Q

What is calcinosis cutis

A

Deposition of calcium hydroxyappitite in skin and soft tissues
Occurs in fingerpads, palms, extensor surfaces of forearms, olecranon and pre-patellar bursa
More common in patients with anti-centromere antibodies and limited

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14
Q

What is acro-osteolysis

A

Resorption of the terminal phalanges due to ischaemia in severe raynauds

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15
Q

What are the two most common lung abnormalities in SSc and what type are they seen in

A

Interstitial lung disease

  • more common with diffuse (65%) and those with Scl-70 antibodies
  • usually NSIP (ground glass changes)
  • more common in afro-americans
  • progression occurs early

Pulmonary artery hypertension

  • limited SSc with anticentromere antibodies
  • occurs in 15% of diffuse also but is due to ILD

30% of scleroderma patients have lung disease

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16
Q

What are GI manifestations of SSc

A

GORD
Periodontal disease
Reduced lower oesophageal motility and delayed gastric emptying
Oesophageal strictures
Gastric natural vascular ectasia
Malabsorption and diarrhoea from bacterial overgrowth
- iron deficiency
Primary biliary cirrhosis ( more in limited)
Wide mouthed colonic diverticulae
Pneumatosis cystodies intestinalis

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17
Q

What us the renal complication of SSc

A

Scleroderma renal crisis
-malignant HTN
- Occurs in 10-15% of patients usually within 4 years of disease onset
Accelerated hypertension and progressive renal failure
- increased risk if antibodies to RNA polymerase III

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18
Q

Treatment of scleroderma renal crisis

A

Short acting ACE inhibitor (increased survival from 15% - 76%)
Avoid steroids as can be associated with development of renal crisis
Dialysis and transplant if required

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19
Q

Cardiac manifestations of SSc

A

Pericardial effusion
Arrythmias and conduction abnormalities
Increased risk of MI
Ventricular dysfunction and valvular insufficiency

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20
Q

Antibodies and their associations in SSc

A
  1. ANA positive in 95%
  2. anti topoisomerase - Scl-70 antibody
    - speckled pattern associated with diffuse and lung fibrosis
  3. anti centromere (ACA) antibody - centromere pattern
    - associated with limited SSc, pulmonary HTN and esophageal disease
  4. anti RNA polymerase 3 antibody
    - nucleolar pattern associated with diffuse, renal and skin involvement and increased risk of malignancy
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21
Q

Management options for skin disease

A

Methotrexate - preferred if also arthritis
Mycophenolate - preferred if also lung involvement
Cyclophosphamide - only use in refractory cases

in diffuse scleroderma, skin disease improves with time without treatment but hand changes remain

Early skin changes (itch, odema) respond to short course steroids and antihistamines
- must use low does steroids due to risk of renal crisis

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22
Q

Treatment of Raynaud’s phenomenon

A 
Calcium channel blockers (first line)
Angiotensin 2 receptor blockers
Alpha-blockers
5 phosphodiesterase inhibitors
SSRIs
Topical nitrates

For severe involvement

  • IV prostaglandin (heals and prevents ischaemic ulceration) - prevents amputation
  • endothelian-1 receptor antagonist (bosentan)
  • digital sympathectomy
23
Q

What is the preferred treatment for SSc ILD

A

Cyclophosphamide
- most likely to benefit if FVC 20% of HRCT shows ground glass changes
Azathioprine may have a role if unable to tolerate

24
Q

Prognosis of diffuse and limited SSc

A

Diffuse
5 year 70%, 10 year 50%
Limited
5 year 90%, 10 year 75%

25
What are the 3 types of systemic sclerosis
Diffuse Limited Sine scleroderma
26
What is mixed connective tissue disease and it's assoc antibody
Overlap syndrome between SSc, RA, SLE + polymoyositis Anti-U1-RNP antibodies are typically positive without other disease associated antibodies Has a good prognosis and good response to steroids
27
What is the characteristic pathology of Sjogrens
Lymphocytic infiltration of exocrine glands - environmental or endogenous Ag triggers an inflammatory response in susceptible individuals - active interferon pathways suggests ongoing activation of the innate immune system
28
What is the diagnostic criteria for Sjogrens
4 of 6 including histopathology - symptoms of ocular dryness - ocular signs of inadequate tear production - symptoms of oral dryness - tests indicating impaired salivary gland function or structure - salivary gland pathology showing foci of lymphocytes - prescence of auto-antibodies (anti Ro/SSA or anti La/SSb)
29
What are some differentials for Sjogrens
``` Prior head and neck irradiation Hep C infection (cryglobulinaemia) AIDs - HIV can cause diffuse infiltrative lymphocytosis syndrome (DILS) which can cause parotid enlargement, renal, lung and GI involvement (Ab neg) Lymphoma Sarcoidosis Graft vs host Anticholinergic, antihistamine use ```
30
Exocrine features of Sjogrens
Keratoconjunctivitis sicca (dry, gritty eyes) Xerostomia (dental caries, gum recession) Salivary gland enlargement Parotid enlargement Vaginal dryness Non allergic rhinitis and sinusitis Antiphospholipid syndrome
31
Laboratory features of Sjogrens
``` Positive ANA, centromere pattern Anti-Ro/SSA Anti-La/SSB Positive rheumatoid factor Antiphospholipid antibodies - anticardiolipin Abs, lupus anticoagulant ```
32
Lab features is SSc
Iron deficiency anaemia from chronic GI loss Macrocyctic anaemia from malabsorption Renal impairment Microangipathic haemolytic anaemia in renal crisis ESR usually normal compared to other rheumatic disorders
33
Some extraglandular features of sjogrens
``` Monoclonal gammopathy Hypergammaglobulinaemia Erythema nodosum, livedo reticiluris Inflammatory myopathy with proximal muscle weakness Interstital lung disease Type 1 (distal) RTA (hypokalaemia) Interstitial nephritis Autoimmune scelrosing pancreatitis Mononeurtitis multiplex (vasculitis) Neuropathy Cognitive impairment ``` This list is not all of them!
34
What is the main malignant risk with Sjogrens
Lymphoma - Lifetime risk 5% - Mean time to development following onset of Sjogren's is 7.5 years
35
What features suggest development of lymphoma in Sjogrens
``` Persistent parotid enlargement Hepatosplenomegaly Purpura Leukopenia Cryoglobulinaemia Monoclonal gammopathy Low C4 ```
36
Treatment of Sjogrens
Symptomatic relief of dryness - artificial tears, water, pilocarpine trial to stimulate secretions Ophthalmology review Arthralgias - NSAIDs, hydroxychloroquine or MTX RTA - bicarbonate Vasculitis or major organ involvement - steroids and immunosuppressive , IVIg - Rituximab has been used with success - TNF alpha inhibitors have not been shown to be successful Secondary Sjogren's - treat primary condition and symptomatic management
37
With what conditions does secondary Sjogren's overlap?
``` SLE RA Scleroderma Polymyositis Polyarteritis nodosa Cryoglobulinaemia ``` - secondary - primary disease features predominate and secondary Sjogren's is less associated with systemic disease
38
What HLA subtype is linked with Sjogren's?
HLA-DR52 - present in 87% of patients with Sjogren's
39
What changes would be seen in a minor salivary gland biopsy in a patient with Sjogren's?
Aggregations of lymphocytes - predominantly CD4+ T lymphocytes (75%) 10% of the lymphocytes are CD5 + B cells that produce IgM and IgG antibodies, often with a monoclonal or oligoclonal pattern Destruction of normal glandular structure - 40-50% remains
40
What is the female: male ratio for Sjogren's?
9:1
41
What is the most common age of onset of Sjogren's
40's - 50's
42
What is the most common type of lymphoma that occurs in Sjogren's?
MALT - can develop in any nonlymphoid tissue infiltrated by periepithelial lymphoid tissue—most commonly the salivary glands, but also the stomach, nasopharynx, skin, liver, kidneys, and lungs
43
What are some complications of pregnancy with Sjogren;s?
Infants born to mother positive for anti-Ro/SSA are at risk of neonatal lupus and heart block. Increased miscarriage and stillbirth rates
44
What is Schirmer's test?
A piece of filter paper is placed in the lower conjunctiva. The amount of moisture absorbed is measured over 5 mins normal > 15mm definite positive
45
What are the key clinical features of Sjogren's?
``` Sicca symptoms - ocular dryness - positive Schirmer test Oral - dryness of mucosa - dental caries - angular cheilosis, candidiasis Parotid swelling - a firm mass should prompt referral for biopsy ? lymphoma Dry skin and hair Alopecia Arthritis (particularly if secondary and RA) Can get renal, respiratory involvement, vasculitis ```
46
What is the leading cause of death in scleroderma?
PAH + ILD once PAH diagnosed - untreated 2 year survival
47
What features suggest the possibility of PAH in scleroderma?
- suspect in patients with DLCO 1.8 (DLCO reduced much more than FVC) Pulmonary artery pressures > 40 - 50 - moderate risk PAP > 50 = high risk
48
What is the work-up if there is suspicion of PAH in scleroderma?
``` FVC:DLCO Echo HRCT (? ILD as a cause of PAH) 6MWT Right heart catheter study - PAP > 25mmHg, wedge pressure ```
49
What is the treatment for PAH in Scleroderma?
Sildenafil Bosnian Warfarin
50
What are the features of renal crisis in Scleroderma?
New onset HTN Microangiopathic haemolytic anaemia Impaired renal function
51
What are the risk factors for developing renal crisis in Scleroderma?
Baseline impaired renal function RNA polymerase III antibodies + Diffuse scleroderma with truncal involvement High dose steroids
52
What are the cardiac features of Scleroderma?
``` Fibrosis (evident on cMRI) Diastolic dysfunction Arrhythmia PAH Pericarditis ``` occurs in 10% of diffuse scleroderma
53
What is the ratio of limited:diffuse scleroderma?
4:1
54
What are the treatments for Scleroderma?
no proven disease suppressing therapy - treatment is organ specific and symptomatic - DMARDS used for skin disease - hand physic important - monitoring for new organ involvement

Rheumatology (11 decks)

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