Ch 3 - Rheumatology: Other Arthritides

Question 1

What is hemochromatosis?

Answer 1

Organ damage and tissue dysfunction secondary to excessive iron stores and the deposition of hemosiderin

Question 2

What is the clinical presentation of hemochromatosis?

Answer 2

Hepatic cirrhosis,
Cardiomyopathy
DM
Pituitary dysfunction
Skin pigmentation
Chronic progressive arthritis

Question 3

What joints are involved in hemochromatosis?

Answer 3

MC in 2nd and 3rd MCP, PIP joints

May affect hips

Question 4

Who is affected by hemochromatosis?

Answer 4

Males 40 to 50 yo

Question 5

What is treatment of hemochromatosis?

Answer 5

Phlebotomy

NSAIDs

Question 6

What is Alkaptonuria (Ochronosis)?

Answer 6

AR d/o causing def in enzyme homogentisic acid oxidase leads to its increase

Question 7

What is Ochronosis?

Answer 7

Alkalinization and oxidation causes darkening of tissue parts

Question 8

Describe the appearance of Ochronosis.

Answer 8

Bluish discoloration of the urine, cartilage, skin, sclera secondary to the accumulation of homogentisic acid

Question 9

Describe the Progressive degenerative arthropathy in Alkaptonuria (Ochronosis)?

Answer 9

– Onset in 4th decade
– Spinal column involvement
– Arthritis of the large joints, chondrocalcinosis, effusions, osteochondral bodies

Question 10

What is Wilson’s disease?

Answer 10

AR d/o causing deposition of copper l/t destruction: 
– Liver> cirrhosis 
– Brain 
– Kidneys
 – Ocular: Kayser–Fleischer rings

Question 11

What joints have OA in Wilson’s disease?

Answer 11

Wrist
MCP
Knees
Spine

Question 12

What is the treatment of Wilson’s disease?

Answer 12

Copper chelation with penicillamine

Dietary restriction

Question 13

What is affected in Gaucher’s disease?

Answer 13

Glucocerebroside accumulates in the reticuloendothelial cells of the spleen, liver, and bone marrow

Question 14

Who is affected in Gaucher’s disease?

Answer 14

Autosomal recessive

Common in Ashkenazi Jews

Question 15

What joints are involved in Gaucher’s disease?

Answer 15

Monoarticular hip and knee degeneration

Question 16

What is the cause of Sarcoidosis?

Answer 16

Disseminated noncaseating granulomas

Question 17

Who is affected by Sarcoidosis?

Answer 17

• 8× more common in blacks

* More females than males

Question 18

What is the clinical presentation of Sarcoidosis?

Answer 18

– Pulmonary 
– Hilar adenopathy 
– Fever, weight loss, fatigue 
– Skin—Lofgren’s syndrome 
– Erythema nodosum

Question 19

Describe arthritis in Sarcoidosis.

Answer 19

Polyarthritis (4-6 joints)

Knees, PIP, MCP, wrists

Question 20

Describe lab work of amyloidosis.

Answer 20

Homogeneous eosinophilic material seen with Congo red dye

Question 21

What is the clinical presentation of amyloidosis?

Answer 21

– Renal disease is primary clinical feature
– Cardiomyopathy
– Median neuropathy

Question 22

Describe arthritis in amyloidosis.

Answer 22

– Pseudoarthritis—periarticular joint inflammation

– Effusions: Arthrocentesis—“shoulder-pad” sign

Question 23

What is hemophilia?

Answer 23

Blood coagulation disorder caused by factor VIII deficiency (classic hemophilia A) or factor IX deficiency (Christmas disease, hemophilia B)

Question 24

What are the causes of hemophilia?

Answer 24

• X-linked recessive d/o> males

* Assoc w/ HIV 2° to transfusions of factor and blood

Question 25

What joints are commonly affected by hemoarthrosis in hemophilia?

Answer 25

Elbow
Knee
Wrist

Question 26

What causes arthritis in hemophilia?

Answer 26

Remaining blood in the joint depositing hemosiderin into the synovial lining> synovial proliferation and pannus formation

Question 27

What is the treatment of hemophilic arthropathy?

Answer 27

– Conservative care (immobilization, rest, ice)
– Factor VIII replacement
– Rehab
– Joint aspiration as a last resort. Blood in the joint acts as a tamponade to prevent further bleeding

Question 28

What is sickle cell disease?

Answer 28

AR d/o w/ elongated crescent sickle shape du/t ABN Hg S protein, causing obstruction of the microvasculature

Question 29

What is the MC MSK complication of sickle cell disease?

Answer 29

Painful crisis
– Abdomen, chest, back
– Large joints from juxta-articular bone infarcts with synovial ischemia

Question 30

What is “hand-foot” syndrome of sickle cell disease?

Answer 30

Dactylitis: painful, nonpitting swelling of the hands and feet

Question 31

What % of patient will develop osteonecrosis?

Answer 31

1/3 of femoral heads

1/4 of humeral heads

Question 32

What causes osteomyelitis in sickle cell disease?

Answer 32

Salmonella

Question 33

What is a Charçot joint?

Answer 33

Chronic, progressively degenerative arthropathy 2/2 sensory neuropathy l/t joint instability and destruction

Question 34

What are causes of a Charçot joint?

Answer 34

“STD”->”SKA”
• Syringomyelia> Shoulder
• Tabes dorsalis (Syphilis) >Knee
• Diabetic Neuropathy> Ankle

Question 35

What is the number one cause of Charçot joint?

Answer 35

Diabetic Neuropathy

Question 36

What are features that Charçot joint share with OA?

Answer 36

Soft tissue swelling
Osteophytes
Joint effusion

Question 37

What are features that Charçot joint does NOT share with OA?

Answer 37

Bony fragments
Subluxation
Periarticular debris

Question 38

What are radiographic findings of Charçot joint?

Answer 38

• Joint destruction
• Hypertrophic osteophytes
• Loose bodies caused by microfractures
• Disorganization of the joint—subluxation and dislocation