Ch 3 - Rheumatology: Other Arthritides Flashcards
What is hemochromatosis?
Organ damage and tissue dysfunction secondary to excessive iron stores and the deposition of hemosiderin
What is the clinical presentation of hemochromatosis?
Hepatic cirrhosis, Cardiomyopathy DM Pituitary dysfunction Skin pigmentation Chronic progressive arthritis
What joints are involved in hemochromatosis?
MC in 2nd and 3rd MCP, PIP joints
May affect hips
Who is affected by hemochromatosis?
Males 40 to 50 yo
What is treatment of hemochromatosis?
Phlebotomy
NSAIDs
What is Alkaptonuria (Ochronosis)?
AR d/o causing def in enzyme homogentisic acid oxidase leads to its increase
What is Ochronosis?
Alkalinization and oxidation causes darkening of tissue parts
Describe the appearance of Ochronosis.
Bluish discoloration of the urine, cartilage, skin, sclera secondary to the accumulation of homogentisic acid
Describe the Progressive degenerative arthropathy in Alkaptonuria (Ochronosis)?
– Onset in 4th decade
– Spinal column involvement
– Arthritis of the large joints, chondrocalcinosis, effusions, osteochondral bodies
What is Wilson’s disease?
AR d/o causing deposition of copper l/t destruction: – Liver> cirrhosis – Brain – Kidneys – Ocular: Kayser–Fleischer rings
What joints have OA in Wilson’s disease?
Wrist
MCP
Knees
Spine
What is the treatment of Wilson’s disease?
Copper chelation with penicillamine
Dietary restriction
What is affected in Gaucher’s disease?
Glucocerebroside accumulates in the reticuloendothelial cells of the spleen, liver, and bone marrow
Who is affected in Gaucher’s disease?
Autosomal recessive
Common in Ashkenazi Jews
What joints are involved in Gaucher’s disease?
Monoarticular hip and knee degeneration
What is the cause of Sarcoidosis?
Disseminated noncaseating granulomas
Who is affected by Sarcoidosis?
- 8× more common in blacks
* More females than males
What is the clinical presentation of Sarcoidosis?
– Pulmonary – Hilar adenopathy – Fever, weight loss, fatigue – Skin—Lofgren’s syndrome – Erythema nodosum
Describe arthritis in Sarcoidosis.
Polyarthritis (4-6 joints)
Knees, PIP, MCP, wrists
Describe lab work of amyloidosis.
Homogeneous eosinophilic material seen with Congo red dye
What is the clinical presentation of amyloidosis?
– Renal disease is primary clinical feature
– Cardiomyopathy
– Median neuropathy
Describe arthritis in amyloidosis.
– Pseudoarthritis—periarticular joint inflammation
– Effusions: Arthrocentesis—“shoulder-pad” sign
What is hemophilia?
Blood coagulation disorder caused by factor VIII deficiency (classic hemophilia A) or factor IX deficiency (Christmas disease, hemophilia B)
What are the causes of hemophilia?
- X-linked recessive d/o> males
* Assoc w/ HIV 2° to transfusions of factor and blood
What joints are commonly affected by hemoarthrosis in hemophilia?
Elbow
Knee
Wrist
What causes arthritis in hemophilia?
Remaining blood in the joint depositing hemosiderin into the synovial lining> synovial proliferation and pannus formation
What is the treatment of hemophilic arthropathy?
– 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
What is sickle cell disease?
AR d/o w/ elongated crescent sickle shape du/t ABN Hg S protein, causing obstruction of the microvasculature
What is the MC MSK complication of sickle cell disease?
Painful crisis
– Abdomen, chest, back
– Large joints from juxta-articular bone infarcts with synovial ischemia
What is “hand-foot” syndrome of sickle cell disease?
Dactylitis: painful, nonpitting swelling of the hands and feet
What % of patient will develop osteonecrosis?
1/3 of femoral heads
1/4 of humeral heads
What causes osteomyelitis in sickle cell disease?
Salmonella
What is a Charçot joint?
Chronic, progressively degenerative arthropathy 2/2 sensory neuropathy l/t joint instability and destruction
What are causes of a Charçot joint?
“STD”->”SKA”
• Syringomyelia> Shoulder
• Tabes dorsalis (Syphilis) >Knee
• Diabetic Neuropathy> Ankle
What is the number one cause of Charçot joint?
Diabetic Neuropathy
What are features that Charçot joint share with OA?
Soft tissue swelling
Osteophytes
Joint effusion
What are features that Charçot joint does NOT share with OA?
Bony fragments
Subluxation
Periarticular debris
What are radiographic findings of Charçot joint?
- Joint destruction
- Hypertrophic osteophytes
- Loose bodies caused by microfractures
- Disorganization of the joint—subluxation and dislocation
