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319. The answer is d. (Mehta, pp 313-316. Fauci, 14/e, pp 1935-1938.) Osteoarthritis most often affects the weight-bearing joints and is associated with obesity or other forms of mechanical stress. It has no systemic manifestations. It is more common in women and onset is usually after the age of 50. Pain often occurs on exertion and is relieved with rest, after which the joint may become stiff. Distal interphalangeal joints may be involved with the production of Heberden nodes. Bouchard nodes are often found at the proximal interphalangeal joint. Crepitus (the sensation of bone rubbing against bone) is often felt on examination of the involved joint. Rheumatoid arthritis is a systemic disease of women under the age of 40. Joint involvement is usually symmetric, involving the proximal interphalangeal and metacarpophalangeal joints. Ninety-five percent of gouty arthritis occurs in men and often involves the great toe. Chondro-malacia patellae or chondromalacia means softening of the cartilage. Patients present with anterior knee pain and tenderness over the undersur-face of the patella. Pain is worse when sitting for long periods of time or when climbing stairs. Psoriatic arthritis is an asymmetric oligoarthritis that involves the knees, ankles, shoulders, or digits of the hands and feet and occurs in 50% of patients with psoriasis.

320. The answer is d. (Fauci, 14/e, p 1877.) Libman-Sacks endocarditis may occur on any valve (not just the tricuspid valve, as previously thought) but rarely causes any valvular insufficiency. It is a nonbacterial endocarditis probably associated with the antiphopholipid antibody syndrome. It is a source of cerebral emboli.

321. The answer is e. (Fauci, 14/e, pp 2186-2189, 2253-2258, 2267, 2274.) Osteogenesis imperfecta is inherited as an autosomal dominant trait and is characterized by brittle bones that often lead to multiple fractures. Other characteristics include blue sclerae, short stature, deformed skull, hearing loss, and dental abnormalities. Osteomalacia (rickets in children) is a disorder of defective mineralization of the organic matrix of the skeleton. It is due to inadequate intake or metabolism of vitamin D. Patients are susceptible to fractures, weakness, disturbances in growth, and skeletal deformities, but the disorder does not affect the eyes. Paget's disease of the bone or osteitis deformans is due to excessive resorption of bone by osteoclasts; patients present after the age of 40 with swelling or deformity of a long bone or enlargement of the skull. Achondroplasia results from a decrease in the proliferation of cartilage in the growth plate and results in dwarfism.

322. The answer is c. (Fauci, 14/e, p 1781.) HLA-B27 diseases are easy to remember with the mnemonic PAIR (Psoriasis, Ankylosing spondylitis, Inflammatory bowel disease, and Reiter syndrome). These are called the seronegative spodylarthropathies. Reiter syndrome preceded by a bacterial infection (Yersinia, Salmonella, or gonococcus) has a high association with a positive HLA-B27. Ankylosing spondylitis has a 90% association with HLA-B27; overall, Reiter syndrome and inflammatory bowel disease (IBD) have an 80% HLA-B27 association. Patients with IBD (Crohn's disease and ulcerative colitis) may develop a nonerosive oligoarthritis of the large peripheral joints that is usually eliminated after controlling the gastrointestinal symptoms. Arthritis is the second most common extraintestinal manifestation in patients with IBD after anemia (anemia is the most common extraintestinal manifestation). NSAIDs must be used with caution in patients with IBD.

323. The answer is b. (Fauci, 14/e, p 1905.) Aortitis in ankylosing spondylitis may cause aortic insufficiency. The AI manifests itself early in the course of the spinal disease and may lead to congestive heart failure.

324. The answer is b. (Fauci, 14/e, pp 1904-1906.) Ankylosing spondylitis (Marie-Strumpell arthritis) is a chronic and progressive inflammatory disease that most commonly affects the spinal, sacroiliac, and hip joints. All patients have symptomatic sacroiliitis. Other symptoms may include uveitis and aortitis. Men in the third decade of life are most frequently affected and there is a strong association with HLA-B27 (90%) in white patients. Patients with advanced disease present with a bent-over posture. A positive Schober test indicates diminished anterior flexion of the lumbar spine. Involvement of the costoveretebral joints limits chest expansion and eye involvement may cause an iritis. Patients with Reiter syndrome may present with a history of conjunctivitis, urethritis, arthritis, and enthesopathy (Achilles tendinitis).

325. The answer is c. (Fauci, 14/e, pp 1401-1403, 1877, 1918.) The patient most likely has antiphospholipid syndrome. Patients with this antibody are at risk for venous and arterial thrombotic events, probably due to antibody reactivity with platelets or endothelial cell phospholipids. Patients often have a history of miscarriages, leg ulcers, Raynaud's phenomenon, and livedo reticularis. Laboratory data often reveals a positive lupus anticoagulant, thrombocytopenia, a prolonged PTT (not corrected by adding normal plasma; a clotting factor deficiency would correct with normal plasma), elevated titers of anticardiolipin antibodies, and an abnormal dilute Russell's viper venom. Takayasu's arteritis ("pulseless disease") is a granulomatous arteritis that affects women more than men. Patients are usually in their fourth decade of life. The disease typically affects the aorta and its major branches, including the arteries that supply the upper extremities. Patients have absent pulses in the upper arm and complain of arm claudication. Livedo reticularis is characterized by reddish or bluish mottling of the extremities and is usually idiopathic and requires no treatment. Livedo reticularis may be secondary to atheroembolism-induced emboli following an intraarterial procedure. Libman-Sacks disease is endocarditis in patients with SLE and may be associated with the antiphospho-lipid antibodies.

326. The answer is b. (Mehta, pp 296-297.) A Baker's cyst occurs in the midline of the popliteal fossa and is often a complication of rheumatoid arthritis. The cyst represents a diverticulum of the synovial sac that protrudes through the joint capsule of the knee. The knee is composed of 12 different bursae. Anserine bursitis occurs with inflammation of the bursa on the medial side of the proximal tibia. There is localized tenderness and swelling over the knee. Prepatellar bursitis is called housemaid's knee (i.e., associated with scrubbing floors) and is characterized by inflammation of the bursa anterior to the patella. Usually, the history supports the diagnosis. Inflammation of the infrapatellar bursa is called clergyman's or carpet-layer's knee. Deep venous thrombosis (DVT) is due to partial or complete occlusion of a vein by a thrombus and may be characterized by a painful, swollen calf or thigh. Occasionally, there is a positive Homan sign (pain with dorsiflexion of the foot), but often a DVT is asymptomatic.

327. The answer is b. (Fitzpatrick, 3/e, pp 358-367, 396, 410.) The patient presents with symptoms suggestive of scleroderma or progressive systemic sclerosis (PSS). This disease, when diffuse, involves the skin, joints, lungs, heart, and gastrointestinal system. Limited systemic sclerosis (lSSc) was formerly known as the CREST syndrome (Calcinosis cutis, Raynaud's phenomenon, Esophageal dysfunction, Sclerodactyly, and Telangiectasia). Raynaud's phenomenon may be associated with tobacco use, medication use (beta-adrenergic blockers) and other diseases such as systemic lupus erythematosus, rheumatoid arthritis, carpal tunnel syndrome, and thromboangiitis obliterans. Dermatomyositis is a systemic disease characterized by a violaceous rash of the eyelids and periorbital areas (heliotrope) and flat, violaceous papules over the knuckles (Gottron sign). The rash seen in ulcerative colitis is pyoderma gangrenosum. These painful ulcers are large and irregular and drain a purulent, hemorrhagic exudate. Sarcoidosis is a systemic disease with skin manifestations, bilateral hilar adenopathy, and pulmonary disease. Patients with sarcoidosis may present with erythema nodosum, which typically takes the form of multiple firm, red, painful, plaques that are bilateral and most frequently distributed on the legs. Musculoskeletal findings in sarcoidosis include arthritis and tenosynovitis.

328. The answer is e. (Fauci, 14/e, pp 1547,1914-1920.) The multisystem disease described in this patient is most likely Henoch-Schonlein purpura (HSP), which is a small-vessel vasculitis that affects mostly children. The purpura and all of the symptoms described are a result of the vasculitis. Histopathology of the vasculitic lesions reveals the deposition of IgA in the walls of the small vessels (postcapillary venules). The mnemonic for HSP is AGAR (Abdominal pain, Glomerulonephritis, Arthralgia, and Rash). The prognosis for Henoch-Schonlein purpura is excellent. Kawasaki's disease (KD) or mucocutaneous lymph node syndrome is uncommon in children over the age of 8 years and is characterized by fever, a desquamating, ede-matous, blotchy-appearing, mucocutaneous erythema, cervical lymphadenitis, and anuerysms of the coronary arteries. It is idiopathic. Wegener's disease and Goodpasture's disease usually have pulmonary involvement. Cryoglobulinemia does cause palpable purpura, abdominal pain, and glomerulonephritis, but it does not cause any gastrointestinal bleeding. Cryoglobulinemia is associated with hepatitis B or C virus.

329. The answer is c. (Fauci, 14/e, pp 1941-1944.) Tophaceous gout is characterized by the finding in synovial fluid of monosodium urate crystals that are needle-shaped and strongly negative birefringent (bright yellow when parallel to the axis). Gouty attacks may be precipitated by trauma, medications that inhibit tubular secretion of uric acid (aspirin, hydrochlorothiazide), surgery, stress, alcohol, and a high-protein diet. The patient may have an accumulation of tophi in and around the joints and earlobe. Radiographs may show "rat bite" erosions. Pseudogout is due to calcium pyrophosphate dihydrate (CPPD) deposition disease; the crystals here are rhomboid-shaped and weakly positive birefringent (blue when parallel to the axis). Calcium oxalate deposition disease is usually seen in patients with end-stage renal disease; calcium phosphate deposition disease causes calcific tendinitis or Milwaukee shoulder.

330. The answer is b. (Fauci, 14/e, pp 1880-1885.) A septic joint will usually produce systemic symptoms such as fever. Osteoarthritis produces a short period of morning stiffness and often affects the distal interphalangeal joints. Chondrocalcinosis is a radiologic finding (destructive arthropathy) associated with pseudogout or CPPD crystals. The patient most likely has rheumatoid arthritis since she meets four of the seven criteria as classified by the American College of Rheumatology:

1. Symmetric polyarthritis for over 3 mo

2. Morning stiffness lasting more than 1 h

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