Weird and wonderful

Question 1

What are the clinical features of Heerfordt’s syndrome (acute presentation of sarcoidosis - 4 things)?

Answer 1

1. Fever
2. Anterior Uveitis
3. Parotid swelling +/- salivary / lacrimal
4. Facial nerve palsy

+/- skin lesions
+/- pulmonary involvement
+/- neurosarcoidosis (meningism/ ophthalmoplegia/pupillary reflex dysfunction)

Question 2

What is Logren’s syndrome (4 things)?

What is it’s prognosis?

Answer 2

1. Hilar lymphadenopathy
2. Erythema nodosum
3. Arthralgia
4. Fever

Good prognosis.

Question 3

What is Anderson-Fabry disease?

Answer 3

• Alpha-galactosidase A deficiency
• Rare X-linked lysosome storage disease
• Abnormal metabolism of spingolipids

Question 4

What are the clinical manifestations of Anderson-Fabry disease (4)?

Answer 4

Anderson-Fabry was a musician that liked C-SHaRP:

CM
Skin (ARA)
HTN
Renal failure
Pain 

ARA = angiokeratomas, reynauds, anhidrosis

Question 5

What is another name for Anderson-Fabry disease?

Answer 5

Angiokeratoma corpus diffusum

due to diffuse skin lesions

Question 6

Going fro top to bottom anatomically, list the ‘8’ levels of IgG4-related disease

Answer 6

1. Hypophysitis
2 Kuffner (chronic sclerosing sialadenitis) / Mikulicz
3. Reidels thyroditis
4. Interstitial pneumonia
5. Retroperitoneal fibrosis
6. Autoimmune pancreatitis
7. Tubulointertitial nephritis / GN
8. Prostatis

Question 7

What is the life-expectancy of the average Australian male and female.

Answer 7

Male = 84yrs
Female = 87yrs (older)

Rough estimate: men 85 / women 90