MIDTERMS: Systemic Sclerosis, Crystal Arthroplasties & Vasculitis Flashcards
Q: What is systemic sclerosis (scleroderma)?
A: A multisystem autoimmune disease characterized by fibrosis, small vessel vasculopathy, and immune system activation.
Q: What does CREST syndrome stand for?Q: What is the most common early symptom of systemic sclerosis?
Calcinosis (calcium deposits in skin).
Raynaud’s phenomenon (vasospasms).
Esophageal dysfunction (acid reflux).
Sclerodactyly (thick, tight skin on fingers).
Telangiectasias (dilated capillaries on skin).
Q: What are the four types of crystal arthropathies?
Gout (Monosodium Urate Crystals).
Calcium Pyrophosphate Deposition Disease (CPPD, Pseudogout).
Calcium Hydroxyapatite Deposition Disease.
Calcium Oxalate Deposition Disease.
Q: What is the most common early symptom of systemic sclerosis?
A: Raynaud’s phenomenon – cold-induced color changes in fingers and toes.
Q: What is the leading cause of mortality in systemic sclerosis?
A: Pulmonary involvement (pulmonary fibrosis and pulmonary hypertension).
Q: What is the most common cardiac complication of systemic sclerosis?
A: Myocardial fibrosis leading to arrhythmias and heart failure.
Q: What is the mainstay of treatment for systemic sclerosis?
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.
Q: What is the most commonly affected joint in gout?
A: First metatarsophalangeal (MTP) joint – “Podagra”.
Q: What are the classic findings in gouty arthritis?
Sudden severe joint pain (often at night).
Red, swollen, and warm joint.
Needle-shaped, negatively birefringent urate crystals in synovial fluid.
Q: What is the first-line treatment for acute gout?
A: NSAIDs (Indomethacin), Colchicine, or Corticosteroids.
Q: What medications are used for chronic gout management?
Xanthine oxidase inhibitors (Allopurinol, Febuxostat) – lower uric acid production.
Uricosurics (Probenecid, Sulfinpyrazone) – increase uric acid excretion.
Q: What is pseudogout?
A: Arthritis caused by calcium pyrophosphate dihydrate (CPPD) crystals.
Q: What are the crystal characteristics in pseudogout?
A: Rhomboid-shaped, weakly positively birefringent.
Q: What is the most common joint affected in pseudogout?
A: Knee joint.
Q: What are the radiographic findings of CPPD disease?
A: Chondrocalcinosis – calcification of cartilage.
Q: What is vasculitis?
A: Inflammation of blood vessels, leading to ischemia and organ damage.
Q: What are the three main categories of vasculitis based on vessel size?
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.
Q: What is the hallmark of Giant Cell Arteritis (GCA)?
A: Temporal headaches, jaw claudication, and vision loss (ophthalmic artery involvement).
Q: What serious complication can occur in GCA?
A: Irreversible blindness due to anterior ischemic optic neuropathy.
Q: What vasculitis affects young women and is known as “pulseless disease”?
A: Takayasu arteritis – affects the aorta and branches, causing limb claudication.
Q: What is the first-line treatment for Giant Cell Arteritis?
A: High-dose corticosteroids (prednisone 60 mg/day) immediately to prevent blindness.
Q: What pediatric vasculitis affects coronary arteries and can cause aneurysms?
A: Kawasaki disease – diagnosed by fever > 5 days + 4/5 of: conjunctivitis, rash, lymphadenopathy, strawberry tongue, extremity changes.
Q: What is the diagnostic criterion for Polyarteritis Nodosa (PAN)?
A: Medium-sized artery inflammation without ANCA antibodies. Commonly affects kidneys, skin, and nerves.
Q: What are the ANCA-associated vasculitides?
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.
Q: What is the first-line treatment for Kawasaki disease?
A: IV immunoglobulin (IVIG) + aspirin to prevent coronary aneurysms.
Q: What is the hallmark finding in Henoch-Schönlein Purpura (HSP)?
A: Palpable purpura, abdominal pain, and arthritis (often post-infection).
