Immune Multisystem Disorders

Question 1

3 main types of autoimmune disorders?

Answer 1

1) Organ specific with specific antigen e.g. Pernicious Anaemia

2) Organ specific with organ specific antigen e.g. 1y Billiary Sclerosis

3) Multi-system disease e.g. RA, SLE, Sjrogens

Question 2

SLE : Main Tissue Involvement?

Answer 2

• Skin → Malar Rash, Photosensitivity, Discoid rash (with atrophic centre)
• Oral Ulcers →
• Joints
• Neurological → Neuro-psychiatric manifestation
• Serositis → e.g pleuritis, abdominal pain
• Libman-Sacks → non-infective endocarditis; Vegitations on valves
• Renal → glomerulonephritis (immune complex deposition in BM)
• Haemtological → Pancytopenia
• Immunological → ANA +ve (specifically dsDNA); Anti-smith (Ab against ribonucleoproteins, most specific but not very sensitive) & Anti-histone (usually drug-related SLE e.g. hydralazine)

Question 3

What does skin biopsy look like in SLE

Answer 3

Question 4

Scleroderma : What are the 2 types?

Answer 4

• Diffuse : Antibodies to DNA topoisomerase (Scl70)
  
  • Truncal involvement is key
• Limited : Anticentromere Ab
  CREST Syndrome

Question 5

CREST Syndrome

Answer 5

Limited Scleroderma
- Calcinosis
- Raynauds (white (vasoconstriction) → blue (cyanosis) → red (hyperemia))
- Esophagel Dysmotility → due to collagen/fibrin deposition
- Sclerodactyly (± nail fold capillary dilatation)
- Telangiectasia

Question 6

Immunoflourescence pattern in Scleroderma?

Answer 6

Nucleolar pattern

Question 7

Immunoflourescence pattern in mixed CTD?

Answer 7

Speckled ANA pattern

Question 8

S/S of Sarcoidosis?

Answer 8

• Joints → arthralgia
• Skin → Nodules/papules; lupus pernio (nose); Erythema Nodosum
• Lungs → Bilateral Hilar Lymphadenopathy or Fibrosis/Infiltration (dry cough)
• Lymphadenopathy → parotid infiltration
• Heart → Epicardium (pericarditis); Myocardium (HF); Endocardium (valvular lesion)
• Eyes → Uveitis, Conjunctivitis
• Neuro → Meningitis, cranial nerve lesions
• Liver → Hepatitis/serosis ; cholestasis

Question 9

Pathophysiology of Sarcoidosis?

Answer 9

Non Caseating granulomous inflammation

Question 10

Ix for Sarcoidosis?

Answer 10

Hypergammgoblulinemia, Raised ACE & hypercalcemia (due to macrophage activation causing vitamin D hydroxylation)

Question 11

Autoantibodies in SLE?

Answer 11

• Anti-dsDNA
  -Anti-smith (ribonucleoproteins)
• Anti-histone (drug related e.g. hydralazine)

Question 12

Libmans Sacks Endocarditis?

Answer 12

• non-infective endocarditis; Vegitations on valves

Question 13

Pathophysiology or Scleroderma?

Answer 13

Fibrosis and excess collagen in skin

Question 14

Polyarteritis Nodosa?

Answer 14

• Inflammation of gut and renal

o Necrotising arteritis -> heals by fibrosis
o Polymorphs, lymphocytes and eosinophils will infiltrate
o Arteritis is focal and sharply demarcated
o Associations:
 Rosary beads appearance on angiogram (aneurysms)
 HBV

Question 15

Granulomatous with polyangitis

Answer 15

o THREE hallmarks:
 Upper respiratory (ENT) – nosebleeds, sinusitis, saddle nose
 Lower respiratory (lungs) – haemoptysis, SOB
 Kidneys – haematuria
o Antibody: C-ANCA (directed against proteinase 3)

Question 16

Churg Strauss / Eosinophilic granulomatosis with polyangitis

Answer 16

o THREE hallmarks:
 Asthma
 Eosinophilia
 Vasculitis
o Antibody: P-ANCA (directed against myeloperoxidase)

Question 17

Temporal Arterities

Answer 17

o Needs an ESR - needs high dose prednisolone
o A temporal artery biopsy is needed for a definitive diagnosis
 This will show narrowing of the lumen and lymphocytic infiltration of the tunica media (not intima)