4. Arthritis of more than three joint areas
5. Involvement of the joints of the hands and wrists; patients may have swan-neck deformity (hyperextension of the proximal interphalangeal joints with compensatory flexion of the distal joint), boutonnière deformity (extension of the distal interphalangeal joint), and ulnar deviation of the digits
6. A positive rheumatoid factor (RF)
331. The answer is b. (Fauci, 14/e, pp 1901-1903, 1914-1917, 1924.) Wegener's granulomatosis involves the upper airways (nasopharynx and sinuses) and the lungs, kidneys, and joints. The diagnosis is made by the clinical picture, a positive antineutrophil cytoplasmic antibody with a cyto-plasmic staining pattern (C-ANCA), and biopsy showing necrotizing granulomas. The disease causes a systemic necrotizing arteritis and is fatal without treatment. The typical history for Churg-Strauss syndrome (allergic angiitis and granulomatosis) is asthma followed by systemic vasculitis with eosinophilia (mnemonic is RAVE: Rhinitis, Asthma, Vasculitis, and Eosinophilia). Lofgren syndrome is a benign form of sarcoidosis that causes bilateral hilar adenopathy, periarthritis of the ankles, and erythema nodosum of the anterior tibial regions of the lower extremities. Sjögren syndrome is a slowly progressive autoimmune disease that primarily affects middle-aged women; it affects the lacrimal and salivary glands, resulting in xerostomia and dry eyes. It may occur alone (primary) or in association with other autoimmune diseases such as rheumatoid arthritis or systemic lupus erythematosus.
332. The answer is a. (Fauci, 14/e, pp 1910-1913,1951.) The most probable diagnosis is polyarteritis nodosa (PAN), a life-threatening vasculitis of the medium-sized vessel that causes visceral ischemia, especially in the gastrointestinal tract. Other systems, such as the genitourinary system (kidney and testes) and neurologic system (mononeuritis multiplex manifesting itself as wristdrop or footdrop), may also be involved. Hepatitis B has been shown to be present in 50% of patients with PAN. Whipple's disease causes a synovitis of the hands, feet, and knees. Often the patient exhibits fever, lymphadenopathy, and signs of malabsorption, such as diarrhea. Whipple's disease is caused by the infectious agent Tropheryma whip-pellii. Osteonecrosis is usually found in patients with sickle cell disease.
333. The answer is b. (Fauci, 14/e, pp 1875-1876.) The American College of Rheumatology criteria for SLE (need 4 of the 11: BRAIN SOAP M.D.) are the following:
1. Blood or hematologic (hemolytic anemia, thrombocytopenia, or lym-phopenia)
2. Renal (proteinuria or casts)
4. Immunologic (+VDRL or anti-dsDNA Ab or anti-Sm Ab or LE prep)
5. Neurologic (seizures or psychosis)
6. Serositis (pericarditis, pleuritis)
7. Oral ulcers
8. Arthritis that is nonerosive and involves more than two joints
10. Malar rash
334. The answer is b. (Fauci, 14/e, pp 1955-1956.) The history and physical examination revealing "tender points" makes fibromyalgia syndrome the most likely diagnosis. This is a disorder predominantly of females; patients complain of insomnia, easy fatigability, and widespread musculoskeletal pain and stiffness. There are up to 18 symmetrical bilateral tender points occurring in the same locations on all patients. Laboratory data is normal in primary fibromyalgia syndrome. 335-336. The answers are 335-b, 336-c. (Fauci, 14/e, pp 1874, 1910, 1921.) The 69-year-old woman has drug-induced lupus. Drugs that may cause lupus are dilantin, procainamide, quinidine, hydralazine, and isoni-azid. Patients are usually older and renal involvement is rare. The 17-year-old woman has 4 of the 11 criteria for systemic lupus erythematosus. Behcet syndrome is a multisystem disorder that involves the eye and causes painful oral and genital ulcerations. The nondeforming arthritis of Behget syndrome affects the knees and ankles. Thromboangiitis obliterans or Buerger's disease is an inflammatory peripheral vascular disease of the upper and lower extremities that usually affects men under the age of 40 who smoke. Patients may complain of extremity claudication or Raynaud's phenomenon. The treatment of Buerger's disease is to quit smoking cigarettes. 337-339. The answers are 337-c, 338-a, 339-d. (Fauci, 14/e, pp 1884, 1896-1901.) Polymyalgia rheumatica affects older patients. They present with weight loss, profound fatigue, and pain and stiffness of the neck, shoulders, thighs, and hips. Physical examination is typically normal. Temporal arteritis may be seen in patients with polymyalgia rheumatica and must always be ruled out. Dermatomyositis is an autoimmune disease that causes proximal muscle weakness that involves the skin; polymyositis spares the skin. Patients with rheumatoid arthritis who develop splenomegaly and neutropenia are said to have Felty syndrome.
This page intentionally left blank.
Was this article helpful?