conditions Flashcards

1
Q

myositis muscle pattern involvment?

A

symmetrical striated muscle involvment

proximal muscles

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2
Q

Extra-muscular signs of dermatomyositis?

A
  • Raynaud’s
  • interstitial lung fibrosis
  • myocardial involvement (myocarditis, arrhythmias).
  • respiratory failure—> diaphragmatic involvment
  • upper esophageous–>swalling affected
  • aspiration pneumonia—> upper eosphageous affected
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3
Q

Atrial Myxomas can also present with this syndrome

A

Atrial Myxomas can present with a vasculitis like syndrome:

ECHO can exclude this!

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4
Q

what is this? where do u see it in?

what other sign r assciated with this diseasE?

A

Myositis plus skin signs:

•Macular rash (shawl sign is +ve if over back and shoulders). •Lilac-purple (heliotrope) rash on eyelids + oedema .

•Nailfold erythema (dilated capillary loops).

•Gottron’s papules: roughened red papules over the knuckles, also seen on elbows and knees

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5
Q

where is Scleroderma renal crisis often seen in?

A

is a serious condition with features of accelerated hypertension, which can lead to renal failure if not treated promptly. It is seen early in the course of disease in patients with diffuse SSc.

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6
Q

-Often referred to as temporal arteritis (TA)

A

GCA

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7
Q

Drugs that trigger an SLE-like syndrome

A

HY PIM

  • Hydralazine (antihypertensive)
  • Procainamide (antiarryhtmatic)
  • Isoniazid (antiTB)
  • Miocycline (antiobiotic)
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8
Q

Median age of onset is 72

A

GCA

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9
Q

what other rheumatoid conditions can develop myositis?

A

SLE and scleroderma

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10
Q

treatment of Giant cell arteritis

A

Prednisolone 60–100 mg PO per day for at least 2 weeks before considering tapering down slowly

  • For acute onset visual symptoms, consider 1g methylprednisolone IV pulse therapy for the 1–3 days
  • Low-dose aspirin therapy to reduce thrombotic risks
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11
Q

SLE

Common symptoms and signs

A
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12
Q

5-10% life time risk of B cell lymphoma!

A

Sjogren’s syndrome

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13
Q

what is Morphoea?

A

Morphoea is a localized form of Scleroderma

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14
Q

What are the primary vasculitides?

A

Small-vessel vasculitis

  1. Microscopic polyangiitis (MPA)
  2. Eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss syndrome)
  3. Granulomatosis with polyangiitis (GPA, Wegener’s granulomatosis)
    • IgA vasculitis (Henoch-Schönlein purpura)

Medium-vessel vasculitis

  1. Polyarteritis nodosa (PAN)
  2. Kawasaki disease (KD)

Large-vessel vasculitis

  1. Takayasu arteritis (TAK)
  2. Giant cell arteritis (GCA)
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15
Q

What are spondyloarthropathies?

what r they?

A

These are a group of conditions that affect the spine and peripheral joints and are associated with the presence of HLA-B27.

SPEAR

  • *- Ankylosing spondylitis (most common) - Enteropathic arthritis
  • Psoriatic arthritis
  • Reactive arthritis**

-enteric arthritis

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16
Q

More common in women and Asian people.

A

Hypermobility Spectrum Disorder

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17
Q

Extra-articular manifestations of Ankylosing spondylitis

A
  1. Anterior uveitis
  2. Aortic incompetence
  3. AV block
  4. Apical lung fibrosis
  5. Amyloidosis
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18
Q

Crest syndrome? associated with?

A

Systemic sclerosis

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19
Q

Raynauds syndrome is associated w/ what conditions?

A

scleroderma

SLE

Dermatomyositis

Polymyositis

Sjogrens syndrome

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20
Q

5 times more common in women

A

Systemic Sclerosis (SSc)

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21
Q

Ix for SLE

A

Most patients have raised ESR or plasma viscosity

Anaemia & leukopenia are common

Antibody test >>Anti-Ro & Anti-La are common

Anti- dsDNA titre rises with disease activity

Antiphospholipid antibodies increase the risk of pregnancy loss and thrombosis

C3 and C4 fall with disease activity

Urinalysis >> vital for detecting renal disease

  • *Skin biopsy >>** can be diagnostic
  • *Renal biopsy >>** can be diagnostic and can help prognosis
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22
Q

Vasculitis Tx?

A

General Measures: Rule out infection, stop offending drug in secondary causes

  • *1st line:** Corticosteroids
  • *2nd Line:** Cytotoxic medications, immunomodulatory, or biologic agents (e.g., cyclophosphamide methotrexate, azathioprine, leflunomide mycophenolate mofetil, rituximab, IVIG.
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23
Q

C3 and C4 fall with disease activity

A

SLE

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24
Q

Pseudogout crystals and type of ppl it effects

risk factor? features, xray finding? Mx

A

Pseudogout is a form of microcrystal synovitis caused by the deposition of calcium pyrophosphate dihydrate crystals in the synovium.

Risk factors

  • haemochromatosis
  • hyperparathyroidism
  • acromegaly
  • low magnesium, low phosphate
  • Wilson’s disease

Features

knee, wrist and shoulders most commonly affected

joint aspiration: weakly-positively birefringent rhomboid-shaped crystals

x-ray: c

in the knee this can be seen as linear calcifications of the meniscus and articular cartilage

Management

  • aspiration of joint fluid, to exclude septic arthritis
  • NSAIDs or intra-articular, intra-muscular or oral steroids as for gout
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25
Q

name Large vessel Vasculitis & Medium Vessel

A

LARGE >>>> GCA & Takayasu arteritis

MEDIUM>>PIK

  • Polyarteritis Nodosa
  • Kawasaki Disease
  • Isolated Central Nervous system vasculitis
26
Q

Absence of Raynaud’s phenomenon makes the diagnosis very unlikely

A

systemic sclerosis

27
Q

What is systemic sclerosis?

A

Multisystem autoimmune disease.
Previously known as scleroderma.
Increased fibroblast activity resulting in abnormal growth of connective tissue which leads to vascular damage and fibrosis.

28
Q

Enteropathic arthritis

2 types of peripheral disease?

A

o Type 1 = oligoarticular, asymmetric & has a correlation w/ IBD flares

o Type 2 = polyarticular symmetrical & less correlation with IBD flares

29
Q

Ractive arthritis symptoms

A
30
Q

teleangiectasis

A

is a condition in which widened venules (tiny blood vessels) cause threadlike red lines or patterns on the skin.

telangiectases form gradually and often in clusters.

They’re sometimes known as “spider veins” because of their fine and weblike appearance

31
Q

well demonstrated on the MRI

A

myositis

32
Q

Antiphospholipid syndrome

A

Hypercoagulble state!!!!

CLOT

Coagulation defect (arterial/venous)

Livedo reticularis

Obstetric (recurrent miscarriage)

Thrombocytopenia.

Thrombotic tendency affects cerebral, renal, and other vessels

Dx: Persistent antiphosphlolipid antibodies with clinical features

Tx: Anticoagulation; seek advice in pregnancy

33
Q

Headache is the most frequent symptom (70%)

A

GCA

34
Q

Osteoporosis tx

A

Vitamin D ± calcium supplementation plus:
1st line: Oral bisphosphonates, or IV if oral not tolerated.
2nd line: Denosumab or teriparatide

35
Q

many ptx have normal inflammatory markers?

A

dermatomyositis & polymyositis

36
Q

ESR and plasma viscosity are raised in most patients but CRP is normal

A

SLE

37
Q

most feared complication of GCA?

A

Permanent visual loss

38
Q

what is Z-score?

A

is a comparison of the patient’s BMD with an age- & gender-matched population.

  • A Z-score <−2 should prompt evaluation for causes of secondary osteoporosis.
39
Q

Causes of positive ANA

A
  • Auto-immune rheumatic diseases e.g. SLE, RA, Sjogren’s sydrome
  • Other auto-immune diseases e.g. ITP
  • Chronic liver disease
  • Chronic infection
  • Malignancy
  • Drug-induced e.g. minocycline
40
Q

Felty’s syndrome

A

RA

41
Q

what genetic predisposition is GCA associated with?

A

HLA-DR24

42
Q

Biopsy is characteristic

A

Sjogren’s syndrome

focal lymphocytic infiltration of exocrine glands.

43
Q

types of Systemic Sclerosis (SSc)?

A

Diffuse Scleroderma & Limited Scleroderma

44
Q

Giant cell arteritis –> inflammation of arteries supplying what areas mainly

A

supplying the

muscles of mastication results in jaw

claudication and tongue discomfort

45
Q

what is this? which condition associated w/?

A

keratoderma blennorrhagica

REactive arthritis

plaques on soles/palms

46
Q

Features of inflammatory back pain

A

IPAIN!

Insidious onset
Pain at night (with improvement on getting up)
Age at onset < 40 Improvement with exercise
No improvement with rest

47
Q

Tx SLE

A
  • Sun protection
  • Advice on healthy lifestyle due to CVD
  • Hydroxychloroquine is helpful for rash and arthralgia
  • Mycophenolate mofetil, azathioprine & rituximab are commonly used too

Short courses of prednisolone for flares

48
Q

Ptx have increased risk of malignancy escpecially in the 2-3 years before and after the diagnosis of it?

A

Dermatomyositis

49
Q

Antibodies in Systemic sclerosis

A
  • Positive ANA in 90% of patients
  • Anti-centromere antibody strongly associated with limited SSc.
  • Scl-70 (topisomerase) and anti RNA polymerase III antibodies strongly associated with diffuse SSc.
50
Q

Sjogren’s syndrome

S & S

A

mnemonic –madfred!

  • Myalgia
  • Arthralgia
  • Dry Mouth
  • Fatigue
  • Raynaud’s phenomenon
  • Enlarged parotids
  • Dry eyes
51
Q

Associated with other autoimmune conditions

A

Sjogren’s syndrome

Coeliac disease, Primary Biliary Cirrhosis, Auto immune thyroid disease.

52
Q

If there is not a dramatic and rapid response to steroids, reconsider the diagnosis.

A

Polymyalgia Rheumatica

53
Q

Reactive arthritis

  • definition
  • presentation
  • other features
  • Ix
A

Sterile synovitis developing after a distant infection either post dysentery (Salmonella / Shigella / Campylobacter) or following urethritis/cervicitis (Chlamydia trachomatis)

- Presentation: few days – 2 weeks post infection, acute asymmetrical lower limb arthritis develops.

- Other features: skin (circinate balanitis, keratoderma blennorrhagica), eye (conjunctivitis, uveitis), enthesitis.

- Ix: serology/microbiology, inflammatory markers raised,

may need joint aspirate to rule out septic/crystal arthritis

54
Q

Tx of Raynauds

complication?

A

All patients should be advised to keep warm & avoid smoking.

1st line: Ca+ channel blockers

PDE 5 inhibitors, and prostacyclins are usually effective.

There is a weak evidence base for angiotensin receptor antagonists, ACE inhibitors, SSRIs, systemic and topical nitrates.

Complications in RP include digital ulcers, severe digital ischaemia and infection.

55
Q

what imaging is useful in DM and scleorderma? why?

A

Nail-fold capillaroscopy is non-invasive

This imaging tool enables evaluation of characteristic structural changes in the peripheral microcirculation

56
Q

what r the risks for osteoporotic fractures

A

FRACTURES

FH, RA, Alcohol, cigarette, thin, UC (IBD), reduced mobility, endocrinopathies, steroids

57
Q

what do we use to capture those at risk with osteoporosis?

A

FRAX TOOL

58
Q

gold standard diagnosis for osteoporosis?

A

Dual eneregy X-ray absorptiometry (DEXA) of lumbar spine and hip

59
Q

Fibromyalgia symp

A

Triad of

  1. poor sleep
  2. fatigue
  3. pain everywhere
60
Q

Ix of Systemic sclerosis

A

Inflammatory markers normal

xray hands>> calcinosis hands

PFT, HRCT

ECO and Ecg

antobodies

61
Q
A

Typical systemic sclerosis face

tapered nose

Pursed mouth