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Rheumatology > Systemic Sclerosis + Sjogrens > Flashcards

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
Q

What are the 3 types of systemic sclerosis

A

Diffuse
Limited
Sine scleroderma

26
Q

What is mixed connective tissue disease and it’s assoc antibody

A

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
Q

What is the characteristic pathology of Sjogrens

A

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
Q

What is the diagnostic criteria for Sjogrens

A

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
Q

What are some differentials for Sjogrens

A 
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
Q

Exocrine features of Sjogrens

A

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
Q

Laboratory features of Sjogrens

A 
Positive ANA, centromere pattern
Anti-Ro/SSA
Anti-La/SSB
Positive rheumatoid factor
Antiphospholipid antibodies - anticardiolipin Abs, lupus anticoagulant
32
Q

Lab features is SSc

A

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
Q

Some extraglandular features of sjogrens

A 
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
Q

What is the main malignant risk with Sjogrens

A

Lymphoma

  • Lifetime risk 5%
  • Mean time to development following onset of Sjogren’s is 7.5 years
35
Q

What features suggest development of lymphoma in Sjogrens

A 
Persistent parotid enlargement
Hepatosplenomegaly
Purpura
Leukopenia
Cryoglobulinaemia
Monoclonal gammopathy
Low C4
36
Q

Treatment of Sjogrens

A

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
Q

With what conditions does secondary Sjogren’s overlap?

A 
SLE
RA
Scleroderma
Polymyositis
Polyarteritis nodosa
Cryoglobulinaemia
  • secondary - primary disease features predominate and secondary Sjogren’s is less associated with systemic disease
38
Q

What HLA subtype is linked with Sjogren’s?

A

HLA-DR52 - present in 87% of patients with Sjogren’s

39
Q

What changes would be seen in a minor salivary gland biopsy in a patient with Sjogren’s?

A

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
Q

What is the female: male ratio for Sjogren’s?

A

9:1

41
Q

What is the most common age of onset of Sjogren’s

A

40’s - 50’s

42
Q

What is the most common type of lymphoma that occurs in Sjogren’s?

A

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
Q

What are some complications of pregnancy with Sjogren;s?

A

Infants born to mother positive for anti-Ro/SSA are at risk of neonatal lupus and heart block.
Increased miscarriage and stillbirth rates

44
Q

What is Schirmer’s test?

A

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
Q

What are the key clinical features of Sjogren’s?

A 
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
Q

What is the leading cause of death in scleroderma?

A

PAH + ILD

once PAH diagnosed
- untreated 2 year survival

47
Q

What features suggest the possibility of PAH in scleroderma?

A
  • 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
Q

What is the work-up if there is suspicion of PAH in scleroderma?

A 
FVC:DLCO
Echo
HRCT (? ILD as a cause of PAH)
6MWT
Right heart catheter study
- PAP > 25mmHg, wedge pressure
49
Q

What is the treatment for PAH in Scleroderma?

A

Sildenafil
Bosnian
Warfarin

50
Q

What are the features of renal crisis in Scleroderma?

A

New onset HTN
Microangiopathic haemolytic anaemia
Impaired renal function

51
Q

What are the risk factors for developing renal crisis in Scleroderma?

A

Baseline impaired renal function
RNA polymerase III antibodies +
Diffuse scleroderma with truncal involvement
High dose steroids

52
Q

What are the cardiac features of Scleroderma?

A 
Fibrosis (evident on cMRI)
Diastolic dysfunction
Arrhythmia
PAH
Pericarditis

occurs in 10% of diffuse scleroderma

53
Q

What is the ratio of limited:diffuse scleroderma?

A

4:1

54
Q

What are the treatments for Scleroderma?

A

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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