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Year 5 Path Histopathology > Immune-related multi-system diseases > Flashcards

Immune-related multi-system diseases Flashcards

1
Q

What are the diagnostic criteria/ clinical presenation of SLE?

A

4 of 11 ACR criteria (SOAP BRAIN MD)
* Serositis
* Oral ulcers
* Arthritis
* Photosensitivity
* Blood disorders (AIHA, ITP, leucopenia)
* Renal involvement ANA +ve
* Immune phenomena (dsDNA, anti-Sm, Antiphospholipid Ab)
* Neuro symptoms
* Malar rash
* Discoid rash

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2
Q

What antibody can be tested for if you suspectd drug-related SLE?

A

Anti-histone

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3
Q

What is the most specific antibody for diagnosis of SLE?

A

Anti-Sm (Anti-smith) antibodies
Only positive in 30% of patients but highly specific

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4
Q

What are the most sensitive antibodies for detection of SLE?

A

ANA (anti dsDNA)

Positive titers of ≥ 1:80 have ∼ 98% sensitivity for SLE

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5
Q

What are the findings on Histology for patients with SLE?
in
1. Bloods
2. Kidney
3. CNS
4. Skin
5.Cardiac

A
  1. LE test –> denaturated nucleus in neutrophils (not done anymore)
  2. Kidney –wire-loop appearance of glomeruli
  3. CNS : small vessel angiopathy
  4. Skin: antibodies on epidermal-dermo junction (picture)
  5. Spleen -. “onion skin” lesions
  6. Heart– Libman-Sack endocarditis (non-infective endocarditis)
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6
Q

What is the pathophysiology of systemic sclerosis

A

2 main things
1. Endothelial changes in blood vessels
2. increased collagen release –> firbrosis of tissue (due to inflammation)

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7
Q

What are the features of limited cutaneous sclerosis?

What are the associated antibodies?

A

CREST syndrome
1. Calcinosis
2. Raynauds
3. esophageal dysmobility
4. sclerodactily
5. telangiectasia

–> anti-centromere antibodies

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8
Q

What is the difference between limited and diffuse systemic sclerosis?

A

Limited cutaneous systemic sclerosis

  • Starts at Skin of feet, lower legs, hands and forearm
  • progresses centrally and can have organ involvement in 10-20 years after onset
  • Anti-centromere antibodies
  • Primary pulmonary hypertension

Diffuse cutaneous systemic sclerosis

  • Rare
  • Wide-spread skin involvement
  • Early involvement of organ damage
  • With interstitial lung disease (rarely pulmonary hypertension)
  • Scleroderma kidney/renal disease –> renal hypertension due to fibrosis and microvascular –> renal hypoperfusion
  • Anti scl70 staining (and others)
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9
Q

What are the histological finidings of limited systemic sclerosis?

A

↑collagen in skin and organs
* “Onion skin” thickening of arterioles [BUZZWORD]

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10
Q

What antibodies are used in diagnosis of limited systemic sclerosis?

A

Anti- centromere

–> speckled nuclear pattern

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11
Q

What antibodies are associated with the diagnosis of diffuse systemic sclerosis?

A

Anti- topoisomerase II (Scl-70)

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12
Q

What is polymyositis and dermatomyositis?

A

Skeletal muscle disorders characterised by progressive muscle weakness and inflammation on muscle biopsy (definitive diagnosis)

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13
Q

What is the aetiology/ association of dermatomyositis or polymyositis?

A

No clear cause but several associations

  1. Polymyositis (PM): cell-mediated cytotoxicity against unidentified skeletal muscle antigens, chiefly affecting the endomysium
  2. Dermatomyositis (DM): idiopathic or paraneoplastic antibody-mediated vasculopathy, associated with malignancies (non-Hodgkin lymphoma; lung, stomach, colorectal, or ovarian cancer )
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14
Q

What is the main clinical presentation of dermatomyositis and polymyositis?

A
  1. progressively worsening proximal myopathy

2. Cutaneous features in dermatomyositis

  • Macular ‘lilac’ heliotrope rash on upper eyelids with periorbital oedema,
  • ash on chest wall, neck (shawl signs) , elbows or knees
  • Gottron’s papules (scaly erythematous raised/thickened plaques on finger joints)
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15
Q

What is sarcoidosis?

A

Multisystem, Connective tissue disease, can involve any organ system, most commonly lung and skin

  • forms non-caseating granulomas.
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16
Q

What is the pathological hallmark of sarcoidosis?

A

non-caseating granulomas

17
Q

What is the epidemiology of sarcoidosis?

A
  • Average age of onset: 30–55 years [1][2]
  • Sex: ♀ > ♂ (2:1)

Prevalence
* Highest in African American and Scandinavian populations
* 2–4 times higher in Black individuals than in non-Hispanic white individuals

18
Q

Explain the changes in calcium seen with sarcoidosis

A

Calcium dysregulation
1. activated pulmonary alveolar macrophages →
2. ↑ 1-alpha hydroxylase expression and activity →
3. ↑ 1,25-dihydroxyvitamin D (calcitriol) →
4. hypervitaminosis D →
5. hyperphosphatemia, hypercalcemia, and, possibly, renal failure

19
Q

What are the clinical signs and symptoms of sarcoidosis?

A

Can caus anythign

  1. Most commonly: lung –> chronic dry cough + hypercalaemia
  2. Night sweats, chest pain

Many many extra-pulmonary manifestations

  • SKIN: erythema nodosum (tender red nodules on shins), lupus pernio (red/purple
    lesions around nose), skin nodules
  • LNs: lymphadenopathy, painless and rubbery
  • JOINTS: arthritis, bone cysts
  • EYES: anterior uveitis → misting of vision and painful red eye; posterior Uveitis → progressive visual loss; uveoparotid fever = bilateral uveitis, parotid enlargement +/- facial nerve palsy (Heerfordt’s Syndrome); keratoconjunctivitis, lacrimal gland enlargement
  • LIVER/SPLEEN: Hepatosplenomegaly
  • BLOOD: Leukopaenia/ anaemia
  • Hypercalcaemia/hypercalciuria → renal calculi + nephrocalcinosis
  • HEART→ dysrhythmias, cardiomyopathy, conduction defects, pericarditis,
  • valvular lesions
  • CNS involvement
  • CONSTITUTIONAL SX: malaise, fever, wt loss, night sweats
20
Q

What are the investigations that should be done in a patient with suspeceted sarcoidosis?

A
  • ↑Ca2+ (ectopic 1-alpha hydroxylase release by activated macrophages)
  • ↑ESR,
  • ↑ACE (due to pulmonary production)
  • Transbronchial biopsy → non caseating granuloma
  • Spirometry might show
21
Q

How are vasculitides classified?

A

Classified by Vessle size
1. Large Vessle
2. Medium Vessle
3. Small Vessle

22
Q

What are the 2 Large Vessel Vasculitides?

A
  1. Giant Cell Arteritis (Temporal arteritis)
  2. Takayasu’s arteritis
23
Q

What are the histological findigns for GCA?

A

Remember to have ESR first

Granulomatous transmural inflammation + giant cells (fused cells) + skip lesions

and immediate treatment with high-dose Prednisolone

24
Q

What are the 2 most important medim vessle Vasculitides?

A
  1. Kawasaki’s disease
  2. Polyarteritis nodosa
25
Q

What is Polyarteritis nodosa?
Which organs are primarily involved?

A

Medium-vessle vasculitis, most commonly in middle- aged adults

  • often associated with renal impirement
  • Associated with Hepatits B (in 30%)
  • can involve any organ, but lung-sparing
  • presents with abdominal pain, muscle, joint pain
  • fever malaise
  • rash ulcers and **nodules (nodosa) **
26
Q

What is the angiographic appearance of polyarterittis nodosa?

A

Microaneurysms on angiography (“string of pearls / rosary bead appearance”)

–> NODOSA = nodules = string of pearls

27
Q

What are the most important Small-vessele vasculitides?

A
  1. cANCA: Granulomatosis with polyangitis (former Wagner’s)
  2. pANCA –> Eosinophic granulomatosis with polyangitis and
  3. pANCA –> Microscopic polyangitis
  4. Henoch Schönlein purpura
28
Q

What is granulomatosis with polyangitis?

What are the organ systems mainly involved?

A

It is a small vessle vasculitis associaetd with cANCA antibodies

Triad of:
(1) Upper resp tract: sinusitis, epistaxis, saddle nose
(2) Lower resp tract: cavitation, pulmonary haemorrhage
(3) Kidneys: crescentic glomerulonephritis → haematuria & proteinuria

29
Q

What is Eosinophilic granulomatosis with polyangitis?

A

pANCA associated small-vessle vasculitis

  • asthma
  • eosinophilia
  • vasculitis

–>to remember: Eosinophils are associated with asthma –> Eosinophilic grnaulomatosis associated with asthma

30
Q

What is amyloidosis?

A

A multi-system connective tissue disease characterised by deposition of misfolded amyloid proteins

31
Q

What are the main sub-types of Amyloidosis?

A
  1. Primary (AL) Amyloidosis
  • most common
  • deposition of Ig light chains
  • associated with multiple myeloma –> production of monoclonal Ig light chains
  1. Secondary (AA) amyloidosis –> chronic inflammation –> production of amyloid A (acute phase protein)
  • e.g. autoimmune disease (60%), RA, ank spond, iBD
32
Q

What are the main clinical/ histological features of amyloidosis?

A

● KIDNEY: nephrotic syndrome = most common presentation (especially seconary)
● HEART: restrictive cardiomyopathy, conduction defects, heart failure,
cardiomegaly
● LIVER/SPLEEN: hepatosplenomegaly
● TONGUE: macroglossia in 10%
● NEUROPATHIES: incl carpal tunnel

33
Q

What histological stains are used to diagnose amyloidosis?

A
  1. Congo Red stain
    2.Appear Apple green birefringence with Congo red stain under polarized light
34
Q

What is Takayasu’s artierits?
What is the main clinical presentation of Takayasu’s arteritis?

A
  • Affects branches of the aortic arch
  • Inflammatory phase → FLAWS
  • Pulseless phase → ”Pulseless” (discreprancy in BP between arms) , claudication, cold hands
  • ↑ in Japanese women
35
Q

What are the investigations that should be done in a patient with suspeceted sarcoidosis?

A
  • ↑Ca2+ (ectopic 1-alpha hydroxylase release by activated macrophages)
  • ↑ESR,
  • ↑ACE (due to pulmonary production)
  • Transbronchial biopsy → non caseating granuloma
  • Spirometry might show

Year 5 Path Histopathology (19 decks)

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