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402: Rheum and derm > Autoimmune connective tissue diseases > Flashcards

Autoimmune connective tissue diseases Flashcards

1
Q

What MSK complication can arising from SLE

A

Osteonecrosis of the femoral head

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2
Q

What hypersensitivity reaction is involved in SLE

A

Type 3
- Immune complexes are deposited in tissue as they fail to clear.

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3
Q

What drugs are associated with lupus

A

Procainamide

Isoniazid

Hydralazine

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4
Q

SLE is more prevalent in which populations?

A

African and Asians

Female> Male (9:1)

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5
Q

Dermatological features of SLE

A

Photosensitive malar rash
- Nasolabia sparing

Discoid lesions

Non-scarring alopecia

Reynaud’s phenomenon

Livedo reticularis

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6
Q

MSK presentation of SLE

A

Migratory joint pain (non-erosive arthritis)
- Knees, wrist, PIPJ

Myalgia

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7
Q

The most common heart manifestations of SLE are…

A

Pericarditis (most common)

Libman Sachs endocarditis ( Nonbacterial thrombotic endocarditis)

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8
Q

The most common renal manifestation of SLE is…

A

Proliferative glomerulonephritis

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9
Q

Neuropsychiatric manifestations of SLE

A

CVA

Mood disorder
- Depression, anxiety

Psychosis

Transver myelitis

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10
Q

Neonatal/ congenital lupus can present with what cardiac manifestation?

A

Complete heart block
- As anti-RO antibodies can cross placenta

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11
Q

The most specific serology for SLE is…

A

Anti-double stranded DNA antibodies

Anti-smith antibodies (ENA)

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12
Q

Antiphospholipid antibodies seen in SLE

A

Anticardiolipin

Lupus anticoagulant

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13
Q

The most sensitive serology for SLE is…

A

Anti-nuclear antibodies (ANA)
- 99% are positive.

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14
Q

What markers can be used to monitor disease activity of SLE

A

ESR

C3, C4 levels
- Lower in acute phase as they are used uo

Anti-dsDNA titres

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15
Q

Pregnant women with SLE have to be given _____ (what and when) to reduce the risk of pre-eclampsia

A

Low dose aspirin from week 12.

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16
Q

Respiratory manifestations of SLE

A

Pleurisy (pleuritic chest pain)

Interstitial fibrosis

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17
Q

First line treatment for non-organ threatening disease in SLE is…

A

Hydroxychloroquine

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18
Q

First line treatment option for organ involvement in SLE is…

A

Cyclophosphamide

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19
Q

Complications of sjogren’s syndrome

A

Eyes
- conjunctivitis
- Corneal ulcers

Oral
- Halitosis
- Dental cavities
- Candida

Vagina
- Vaginitis
- Candidiasis

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20
Q

Extra-glandular presentation of Sjogren’s [5]

A

Joint pain/ arthritis

Vasculitis

Neuropathy

Interstitial lung disease

Nephritis

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21
Q

Causes of secondary Sjogren’s syndrome

A

Rheumatoid arthritis

SLE

systemic sclerosis

Polymyositis

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22
Q

Sjogren’s syndrome is associated with a 40-60 fold increase in…

A

Lymphoid malignancy

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23
Q

Serological diagnosis of Sjogren’s

A

ANA antibodies (70% sensitivity)
- Anti-Ro
- Anti-La

RF

Hypergammaglobulinaemia

Low C4

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24
Q

Histological diagnosis of Sjogren’s syndrome

A

Salivary lymph gland biopsy
- Shows focal lymphocytic infiltrates

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25
Q

Which two ENA antibodies are associated with Sjogren’s syndrome

A

Anti-Ro (SSA)

Anti-La (SSB)

26
Q

Schirmer’s test ________ is suggestive of insufficient tear production, as seen in Sjogren’s syndrome

A

<10mm

27
Q

Management of Sjogren’s syndrome

A

Artifical saliva and tears

Hydroxychloroquine

28
Q

Features of CREST syndrome

A

C- Calcinosis

R- Raynaud’s phenomenon

E- Esophageal dysmotility

S- Sclerodactyly

T- Telangectasia

29
Q

Types of systemic sclerosis

A

Limited cutaneous

Diffuse cutaneous

30
Q

Limited cutaneous sclerosis affects (distal/proximal) region and has a _______ progression

A

Affects distal regions and has slow progression

31
Q

What type of systemic sclerosis presents with CREST syndrome

A

Limited cutaneous sclerosis

32
Q

Diffuse cutaneous sclerosis affects (distal/proximal) region and has a _______ progression

A

Affects the proximal region and shows a rapid progression

33
Q

What type of systemic sclerosis has a high risk of renal crisis

A

Diffuse systemic sclerosis

34
Q

Serology diagnosis for diffuse cutaneous sclerosis

A

ANA antibodies

Anti-scl 70

Anti-RNA polymerase III antibodies

35
Q

Serology diagnosis for limited cutaneous sclerosis

A

ANA antibodies

Anti-centromere antibodies

36
Q

Elevated ___________ is strongly predictive of a _______ in diffuse cutaneous sclerosis

A

Anti-RNA polymerase III antibodies, strongly predictive of a renal crisis.

37
Q

Raynaud’s phenomenon can be managed using…

A

CCB- Nifedipine

38
Q

ILD in systemic sclerosis can be managed using…

A

High dose corticosteroid + cyclophosphamide

39
Q

Scleroderma renal crisis can be managed using…

A

ACE inhibitor

40
Q

Types of idiopathic inflammatory myositis

A

Inclusion body myositis

Dermatomyositis

Polymyositis

41
Q

Inclusion body myositis presents with…

A

Asymmetrical proximal limb weakness + exercise intolerance

Diminished deep tendon reflexes

Dysphagia

42
Q

Inclusion body myositis presents with _________ muscle weakness, whereas polymyositis presents with _______ muscle weakness

A

IBM= asymmetrical proximal weakness

Polymyositis= symmetrical proximal weakness.

43
Q

Polymyositis features

A

Symmetrical muscle weakness

Bulbar and intercostal muscle weakness

44
Q

Dermatomyositis features

A

Dermatological
- Photosensitive, V-shaped rash (anterior chest)
- Heliotrope rash on eyelids

Systemic
- Fever, arthalgia, weight loss

Cardiac
- Arrhythmias, DCM

Gottron’s papules

45
Q

Gottron’s papules are associated with ______

A

Dermatomyositis

46
Q

In myositis, an electromyography would show…

A

Polyphasic action potentions

47
Q

Myositis will show elevated muscle enzymes, which include…

A

Creatinine kinase

ALT

LDH

48
Q

In myositis, serological investigations show elevated…

A

Anti-nuclear antibodies

Anti-synthase antibodies

49
Q

Definite diagnosis of myositis involves…

A

Muscle biopsy and history

50
Q

Patients diagnosis with myositis have an increased risk of _______ so must be screened

A

Malignancies

51
Q

Patients with myositis have an increase risk of which malignancies

A
  • Breast
  • Lung
  • Ovary
52
Q

_______ is used for the initial pharmacological management of myositis

A

Corticosteroids (prednisolone)

53
Q

Drug causes of erythema nodosum

A

Penicillin

COCP

Phenytoin

Sulphonamides

54
Q

Treatment of erythema nodosum

A

NSAIDs

55
Q

Investigations for sarcoidosis

A

Rule out TB
- Mantoux tuberculin test

Bloods
- Bone profile= raised calcium and vit D
- Raised serum ACE
- LFTs- abnormal if liver affected

Serology
- Raised interleukin-2 receptor

ECG

Imaging
- Chest X-ray
- CT

Respiratory function test

Lymph node biopsy and histology
- Non-caseating granuloma with epitheliod cells.

56
Q

CXR in sarcoidosis will show…

A

Bilateral hilar lymphadenopathy

57
Q

What group of people are most commonly affected by sarcoidosis?

A

Age 20-40

Afro-caribbean

Women

58
Q

First-line management of sarcoidosis

A

Oral corticosteroids

59
Q

Second line management in sarcoidosis

A

DMARDs
- Methotrexate
- Azathioprine
- Leflunomide
- Mycophenolate

60
Q

What are the features of Lofgren’s syndrome

A

Seen in sarcoidosis:

  • Erythema nodosum
  • Bilateral hilar lymphadenopathy
  • Polyarthralgia
61
Q

What are the features of Heerfordt’s syndrome

A

Seen in sarcoidosis:

  • Submandibular/ parotid gland inflammation
  • Uveitis
  • CN7 palsy

402: Rheum and derm (12 decks)

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