MIDTERMS: Systemic Sclerosis, Crystal Arthroplasties & Vasculitis

Question 1

Q: What is systemic sclerosis (scleroderma)?

Answer 1

A: A multisystem autoimmune disease characterized by fibrosis, small vessel vasculopathy, and immune system activation.

Question 2

Q: What does CREST syndrome stand for?Q: What is the most common early symptom of systemic sclerosis?

Answer 2

Calcinosis (calcium deposits in skin).

Raynaud’s phenomenon (vasospasms).

Esophageal dysfunction (acid reflux).

Sclerodactyly (thick, tight skin on fingers).

Telangiectasias (dilated capillaries on skin).

Question 3

Q: What are the four types of crystal arthropathies?

Answer 3

Gout (Monosodium Urate Crystals).

Calcium Pyrophosphate Deposition Disease (CPPD, Pseudogout).

Calcium Hydroxyapatite Deposition Disease.

Calcium Oxalate Deposition Disease.

Question 4

Q: What is the most common early symptom of systemic sclerosis?

Answer 4

A: Raynaud’s phenomenon – cold-induced color changes in fingers and toes.

Question 4

Q: What is the leading cause of mortality in systemic sclerosis?

Answer 4

A: Pulmonary involvement (pulmonary fibrosis and pulmonary hypertension).

Question 7

Q: What is the most common cardiac complication of systemic sclerosis?

Answer 7

A: Myocardial fibrosis leading to arrhythmias and heart failure.

Question 7

Q: What is the mainstay of treatment for systemic sclerosis?

Answer 7

Symptomatic management, including:

ACE inhibitors for renal crisis.

Proton pump inhibitors (PPIs) for GERD.

Calcium channel blockers for Raynaud’s phenomenon.

Immunosuppressants (cyclophosphamide, mycophenolate) for lung disease.

Question 9

Q: What is the most commonly affected joint in gout?

Answer 9

A: First metatarsophalangeal (MTP) joint – “Podagra”.

Question 11

Q: What are the classic findings in gouty arthritis?

Answer 11

Sudden severe joint pain (often at night).

Red, swollen, and warm joint.

Needle-shaped, negatively birefringent urate crystals in synovial fluid.

Question 11

Q: What is the first-line treatment for acute gout?

Answer 11

A: NSAIDs (Indomethacin), Colchicine, or Corticosteroids.

Question 12

Q: What medications are used for chronic gout management?

Answer 12

Xanthine oxidase inhibitors (Allopurinol, Febuxostat) – lower uric acid production.

Uricosurics (Probenecid, Sulfinpyrazone) – increase uric acid excretion.

Question 12

Q: What is pseudogout?

Answer 12

A: Arthritis caused by calcium pyrophosphate dihydrate (CPPD) crystals.

Question 12

Q: What are the crystal characteristics in pseudogout?

Answer 12

A: Rhomboid-shaped, weakly positively birefringent.

Question 12

Q: What is the most common joint affected in pseudogout?

Answer 12

A: Knee joint.

Question 13

Q: What are the radiographic findings of CPPD disease?

Answer 13

A: Chondrocalcinosis – calcification of cartilage.

Question 17

Q: What is vasculitis?

Answer 17

A: Inflammation of blood vessels, leading to ischemia and organ damage.

Question 18

Q: What are the three main categories of vasculitis based on vessel size?

Answer 18

Large vessel vasculitis – Giant cell arteritis, Takayasu arteritis. Medium vessel vasculitis – Polyarteritis nodosa, Kawasaki disease. Small vessel vasculitis – ANCA-associated vasculitis (GPA, MPA, EGPA), Henoch-Schönlein purpura.

Question 19

Q: What is the hallmark of Giant Cell Arteritis (GCA)?

Answer 19

A: Temporal headaches, jaw claudication, and vision loss (ophthalmic artery involvement).

Question 19

Q: What serious complication can occur in GCA?

Answer 19

A: Irreversible blindness due to anterior ischemic optic neuropathy.

Question 19

Q: What vasculitis affects young women and is known as "pulseless disease"?

Answer 19

A: Takayasu arteritis – affects the aorta and branches, causing limb claudication.

Question 19

Q: What is the first-line treatment for Giant Cell Arteritis?

Answer 19

A: High-dose corticosteroids (prednisone 60 mg/day) immediately to prevent blindness.

Question 20

Q: What pediatric vasculitis affects coronary arteries and can cause aneurysms?

Answer 20

A: Kawasaki disease – diagnosed by fever > 5 days + 4/5 of: conjunctivitis, rash, lymphadenopathy, strawberry tongue, extremity changes.

Question 20

Q: What is the diagnostic criterion for Polyarteritis Nodosa (PAN)?

Answer 20

A: Medium-sized artery inflammation without ANCA antibodies. Commonly affects kidneys, skin, and nerves.

Question 20

Q: What are the ANCA-associated vasculitides?

Answer 20

Granulomatosis with polyangiitis (GPA, Wegener’s) – c-ANCA positive, sinus/lung/kidney involvement. Microscopic polyangiitis (MPA) – p-ANCA positive, renal involvement. Eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss) – eosinophilia, asthma, neuropathy.

Question 20

Q: What is the first-line treatment for Kawasaki disease?

Answer 20

A: IV immunoglobulin (IVIG) + aspirin to prevent coronary aneurysms.

Question 20

Q: What is the hallmark finding in Henoch-Schönlein Purpura (HSP)?

Answer 20

A: Palpable purpura, abdominal pain, and arthritis (often post-infection).