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TABLE IV-114 Risk Factors for Active Tuberculosis Among Persons Who Have Been Infected With Tubercle Bacilli 27 page

IX-28. The answer is A. (Chap. 324) Although Sjögren’s syndrome most commonly affects the eyes and mouth, there are a number of common extraglandular sites of involvement. The most common is arthritis


or arthralgias that complicated up to 60% of cases. Raynaud’s phenomenon is the second most common extraglandular site. Lung involvement and vasculitis are found in less than 20% of patients. Lymphoma, though a concerning and highly morbid complication, is relatively rare, affecting only 6% of patients with Sjögren’s syndrome.

IX-29. The answer is B. (Chap. 324) The patient in this vignette is presenting with severely dry eyes and mouth in the presence of autoantibodies to Ro and La (SS-A and SS-B, extractable nuclear and cytoplasmic antigens) consistent with the diagnosis of Sjögren’s syndrome. This autoimmune disorder is associated with the lymphocytic infiltration of exocrine glands that results in decreased tear and saliva production as the most prominent symptoms. Sjögren’s syndrome affects women nine times more frequently than men and usually presents in middle age. Other autoimmune diseases often have associated xerostomia and dry eyes (secondary Sjögren’s syndrome). High titers of antibodies to Ro and La are associated with longer disease duration, salivary gland enlargement, and the development of extraglandular involvement, especially cutaneous vasculitis and demyelinating syndromes. One-third of patients with Sjögren’s syndrome have extraglandular involvement of the disease, most commonly in the lungs and kidneys. In this patient with acidemia and hypokalemia, the possibility of renal disease due to Sjögren’s syndrome should be considered. Interstitial nephritis is a common manifestation of Sjögren’s syndrome in the kidneys. Distal (type I) renal tubular acidosis is also frequent, occurring in 25% of individuals with Sjögren’s syndrome. Diagnosis could be confirmed by obtaining urine electrolytes to demonstrate a positive urine anion gap. Renal biopsy is not necessary. Treatment does not require immunosuppression as the acidemia can be treated with bicarbonate replacement. Diarrhea could cause similar electrolyte abnormalities with a non–anion gap acidosis, but the patient would be symptomatic. Furthermore, gastrointestinal symptoms do not commonly occur in Sjögren’s syndrome. Hypoaldosteronism is associated with a type IV renal tubular acidosis that results in hyperkalemia and a non–anion gap acidosis. Renal compensation for respiratory alkalosis should not result in hypokalemia. Purging in anorexia nervosa could result in hypokalemia and increased risk of dental caries, but it would be associated with metabolic alkalosis rather than acidosis.

IX-30. The answer is D. (Chap. 324) Lymphoma is well known to develop specifically in the late stage of Sjögren’s syndrome. Common manifestations of this malignant condition include persistent parotid gland enlargement, purpura, leukopenia, cryoglobulinemia, and low C4 complement levels. Most of the lymphomas are extranodal, marginal zone B cell, and low grade. Low-grade lymphomas may be detected incidentally during a labial biopsy. Mortality is higher in patients with concurrent B symptoms (fevers, night sweats, and weight loss), a lymph node mass greater than 7 cm, and a high or intermediate histologic grade.



IX-31. The answer is D. (Chap. 325) Ankylosing spondylitis is closely correlated with the presence of the histocompatibility antigen HLA-B27. In North American whites, the prevalence of B27 is 7%, but in patients with ankylosing spondylitis it is 90%. Not all persons with B27 develop ankylosing spondylitis; the disease is only present in 1–6% of B27-positive individuals.

IX-32. The answer is A. (Chap. 325) Although the most serious spine complication of ankylosing spondylitis is fracture, there are a number of important extraarticular manifestations. Anterior uveitis is the most common, occurring in 40% of patients with ankylosing spondylitis. Inflammatory bowel disease has been reported to be frequently present. Less common complications include aortic insufficiency, third-degree heart block, pulmonary nodules and upper lobe fibrosis, cardiac dysfunction,


retroperitoneal fibrosis, prostatitis, and amyloidosis.

IX-33. The answer is B. (Chap. 325) Nonsteroidal anti-inflammatory drugs are the first line of pharmacologic therapy for ankylosing spondylitis, of which this patient has a classic presentation. These agents have been shown to reduce pain and tenderness and increase mobility. There is even some evidence that they slow disease progression. Given their proven efficacy, tolerability, and safety, they remain first-line therapy. Anti–TNF-α agents have been reported to have dramatic effects in ankylosing spondylitis, with infliximab, etanercept, adalimumab, and golimumab having published reports of success. Because of their potential side effects, including serious infections, hypersensitivity reactions, and others, these agents should be reserved for patients who fail therapy with NSAIDs.

IX-34. The answer is E. (Chap. 325) Reactive arthritis refers to an acute, nonpurulent arthritis that occurs after an infection elsewhere in the body. Often presenting with lower joint inflammatory arthritis occurring 1–4 weeks after a diarrheal episode, reactive arthritis may also include uveitis or conjunctivitis, dactylitis, urogenital lesions, and characteristic mucocutaneous lesions such as keratoderma blennorrhagica. The most common organism associated with reactive arthritis is Shigella species, although Yersinia, Chlamydia, and, to a much lesser extent, Salmonella and Campylobacter have been described.

IX-35. The answer is D. (Chap. 325) The patient has a spondyloarthritic syndrome with enthesitis and sacroiliitis associated with inflammatory bowel disease. This is a common association called enteropathic arthritis. Generally, enteropathic arthritis responds well to suppression of gastrointestinal disease using anti–TNF-α agents such as infliximab. Other treatments for inflammatory bowel disease are also effective in the resolution of arthritis, such as glucocorticoids and sulfasalazine. NSAIDs may be helpful, but there is significant concern for the precipitation of flares of inflammatory bowel disease and precipitation of ulcers.

IX-36. The answer is E. (Chap. 325) Whipple’s disease is a rare chronic bacterial infection of the gastrointestinal tract most commonly affecting middle-aged men. Arthritis is a common early manifestation of the disease, with arthritis predating gastrointestinal symptoms by 5 years or more. Large and small joints may be affected and sacroiliitis is common. Arthritis is often migratory and lasts several days with spontaneous recovery. Synovial fluid is generally inflammatory. Radiographs rarely show joint erosions, though sacroiliitis may be demonstrated. Diagnosis is often made by PCR amplification of genetic material from Tropheryma whippelii in biopsied material, most commonly the gut.

IX-37. The answer is D. (Chap. 325) Although anti–TNF-α drugs such as infliximab are potent and relatively safe, several types of side effects are not rare. These include serious infections such as disseminated tuberculosis, fungal (histoplasmosis, aspergillus, pneumocystis) and bacterial (legionella, pneumococcus) infections, hematologic disorders such as pancytopenia, demyelinating disorders, systemic lupus erythematosus-related autoantibodies, and potential risk of lymphoma. Additionally, clinical features such as exacerbation of congestive heart failure, hypersensitivity infusion or injection site reactions, and severe liver disease have been described. There are some isolated reports of hypersensitivity pneumonitis, but these are confounded by coadministration of known pulmonary toxic agents.


IX-38. The answer is B. (Chap. 325) Enthesopathy or enthesitis is the term used to describe inflammation at the site of tendinous or ligamentous insertion into bone. This type of inflammation is seen most frequently in patients with seronegative spondyloarthropathies and various infections, especially viral infections. The other definitions apply to other terms used in the orthopedic and rheumatic examination. Subluxation is the alteration of joint alignment so that articulating surfaces incompletely approximate each other. Synovitis refers to the inflammation of the periarticular membrane lining the joint capsule. Inflammation of a saclike cavity near a joint that decreases friction is the definition of bursitis. Finally, crepitus is a palpable vibratory or crackling sensation elicited with joint motion.

IX-39. The answer is C. (Chap. 325) This patient complains of symptoms consistent with a diagnosis of fibromyalgia. Patients with fibromyalgia frequently complain of diffuse body pain, stiffness, paresthesias, disturbed sleep, easy fatigability, and headache. The prevalence of fibromyalgia is approximately 3.4% of females and 0.5% of males. This disorder is thought to represent a disturbance of pain perception. Disturbed sleep with a loss of stage 4 sleep has been implicated as a factor in the pathogenesis of the disease. Serotonin levels in the cerebrospinal fluid have also commonly been seen and may play a role in the pathogenesis. A diagnosis of fibromyalgia is based on the American College of Rheumatology criteria, which combine symptoms and physical examination. The patient must exhibit diffuse pain in all areas of the body with tenderness to palpation at 11 of 18 designated tender point sites. These sites include the occiput, trapezius, cervical spine, lateral epicondyles, supraspinatus muscle, second rib, gluteus, greater trochanter, and knee. Digital palpation should be performed with a moderate degree of pressure. Examination of the joints shows no evidence of inflammatory arthropathy. There are no laboratory tests that are specific for the diagnosis. Positive antinuclear antibodies may be seen, but at the same frequency as in the normal population. HLA-B27 is found in 7% of the white population, but only 1–6% of people with HLA-B27 will develop ankylosing spondylitis. Radiograms are normal in these patients.

IX-40. The answer is D. (Chap. 325) This patient shows the typical features of psoriatic arthritis. Five to ten percent of patients with psoriasis will develop an arthritis associated with the rash. In 60–70% of cases, the rash precedes the diagnosis. However, another 15–20% of patients will have joint complaints as the presenting symptom of their psoriasis. The disease typically begins in the fourth or fifth decade of life. Psoriatic arthritis has varied joint presentations with five commonly described patterns of joint involvement: (1) arthritis of the distal interphalangeal (DIP) joints, (2) asymmetric oligoarthritis, (3) symmetric polyarthritis similar to RA, (4) axial involvement, and (5) arthritis mutilans with the typical “pencil in cup” deformity seen on hand radiography. Erosive joint disease ultimately develops in almost all these patients, and most of them become disabled. Nail changes are prominent in 90% of patients with psoriatic arthritis. Changes that are frequently seen include pitting, horizontal ridging, onycholysis, yellowish discoloration of the nail margins, and dystrophic hyperkeratosis. The diagnosis of psoriatic arthritis is primarily clinical. Thus, in patients with joint symptoms that precede the onset of rash, the diagnosis is frequently missed until dermatologic or nail changes develop. A family history of psoriasis is important to ascertain in any patient with an undiagnosed inflammatory polyarthropathy. The differential diagnosis of DIP arthritis is short; only osteoarthritis and gout are commonly seen in these joints. Radiography may show typical changes, particularly in patients with arthritis mutilans. Treatment is directed at both the rash and the joint disease simultaneously. Anti–TNF-α therapy has recently been shown to be helpful for both the dermatologic and joint manifestations of disease. Other treatments include methotrexate, sulfasalazine,


cyclosporine, retinoic acid derivatives, and psoralen plus ultraviolet light.

IX-41. The answer is E. (Chap. 326) Although the molecular pathology of most vasculitic syndromes is poorly understood, the deposition of immune complexes is commonly thought to play an important role in vasculitis associated with Henoch-Schönlein purpura, cryoglobulinemic vasculitis associated with hepatitis C, serum sickness and cutaneous vasculitic syndromes, and polyarteritis nodosa–like vasculitis associated with hepatitis B. Granulomatosis with polyangiitis (Wegener’s), Churg-Strauss, and microscopic polyangiitis are thought to be due to production of antineutrophilic antibodies. Pathogenic T lymphocyte responses are also implicated in giant cell arteritis, Takayasu’s arteritis, granulomatosis with polyangiitis (Wegener’s), and Churg-Strauss syndrome.

IX-42. The answer is E. (Chap. 326) ANCAs are antibodies directed at proteins in the cytoplasmic granules of neutrophils and monocytes. cANCA, or cytoplasmic ANCA, is directed against proteinase-3, a proteinase present in neutrophil azurophilic granules. More than 90% of patients with granulomatosis with polyangiitis (Wegener’s) will be cANCA positive. Perinuclear ANCA or pANCA refers to a more localized perinuclear staining pattern and antibodies are most commonly directed against myeloperoxidase, though other antigens have been described. pANCA has been reported in variable percentages in microscopic polyangiitis, Churg-Strauss syndrome, and granulomatosis with polyangiitis (Wegener’s) and its variants. Additionally, pANCA staining that is not due to antimyeloperoxidase antibodies has been described in a number of other conditions including rheumatic and nonrheumatic autoimmune disease and inflammatory bowel disease.

IX-43. The answer is D. (Chap. 326) The patient presents with classic symptoms for granulomatosis with polyangiitis (Wegener’s), also known as granulomatosis with polyangiitis. The average age of diagnosis is 40 years and there is a male predominance. Upper respiratory symptoms often predate lung or renal findings and may even present with septal perforation. The diagnosis is made by demonstration of necrotizing granulomatous vasculitis on biopsy. Pulmonary tissue offers the highest yield. Biopsy of the upper airway usually shows the granulomatous inflammation but infrequently shows vasculitis. Renal biopsy may show the presence of pauci-immune glomerulonephritis.

IX-44. The answer is C. (Chap. 326) The patient has a classic presentation for giant cell arteritis with associated polymyalgia rheumatica including headache, jaw claudication, and visual disturbances. Her age makes this diagnosis highly likely as well. The diagnosis is confirmed by temporal artery biopsy; however, in the presence of visual symptoms, initiation of therapy should not be delayed pending a biopsy, as the biopsy may be positive even after approximately 14 days of glucocorticoid therapy. Delay in therapy risks irreversible visual loss. Additionally, a dramatic response to therapy may lend further support to the diagnosis. The primary therapy is prednisone at 40–60 mg daily for 1 month with gradual tapering. Although erythrocyte sedimentation rate is nearly universally elevated, it is not specific for the diagnosis. Temporal artery ultrasound may be suggestive but is not diagnostic.

IX-45. The answer is C. (Chap. 326) The most common manifestations of cryoglobulinemic vasculitis are cutaneous vasculitis, arthritis, peripheral neuropathy, and glomerulonephritis. The demonstration of circulating cryoprecipitates is a critical component of the diagnosis, and often rheumatoid factor can be found as well. Because hepatitis C infection is present in the vast majority of patients with cryoglobulinemic vasculitis, infection should be sought in all patients with this clinical syndrome.


IX-46. The answer is E. (Chap. 326) This patient has polyarteritis nodosa associated with hepatitis B infection. Polyarteritis nodosa (PAN) is a small- and medium-vessel vasculitis that classically involves the muscular mesenteric and renal arteries. Pulmonary arteries are spared. Classic PAN is a rare disease, but its exact prevalence is unknown because reported cases frequently also include other vasculitides such as microscopic polyangiitis. Prior to the Chapel Hill Consensus Conference of 1992, microscopic polyangiitis and PAN were considered as the same disease, but it has been recognized that these are two separate diseases with different serologic markers and vascular predilection. Clinical manifestations of PAN are commonly vague, and often patients have been ill for several months prior to diagnosis. Symptoms include fatigue, weight loss, abdominal pain, headache, and hypertension. The pathologic lesion of PAN is necrotizing inflammation of the small- and medium-sized muscular arteries, and diagnosis relies on demonstration of this lesion on biopsy. However, in the absence of easily obtainable tissue, the presence of multiple aneurysmal dilatations on mesenteric angiogram is highly suggestive of PAN in the appropriate clinical setting. There are no serologic tests that are diagnostic of PAN. It is rare to have positive antibodies to pANCA or cANCA in PAN. Interestingly, 30% of cases of PAN are associated with active hepatitis B infection, as in this patient, and it is thought that circulating immune complexes may play a role in the pathogenesis of this disease. Unlike PAN, microscopic polyangiitis (MPA) involves venules and capillaries in addition to small arteries. The histopathologic lesion of MPA is a necrotizing vasculitis that is pauci immune with minimal deposition of immune complexes. Typical presenting features are rapidly progressive glomerulonephritis and pulmonary hemorrhage, which are distinctly uncommon features of PAN. Antimyeloperoxidase antibodies (pANCA) are frequently present. Mixed cryoglobulinemia is a small-vessel vasculitis most often associated with hepatitis C infection. Skin involvement with leukocytoclastic vasculitis and palpable purpura are the most common presenting features. Proliferative glomerulonephritis is present in 20–60% of individuals and is the most common cause of morbidity. Ischemic colitis typically presents with abdominal pain out of proportion to the examination as in this case, but the mesenteric angiogram would show atherosclerotic narrowing rather than aneurysmal dilatation. Hepatocellular carcinoma is not associated with vasculitis and typically presents with vague abdominal pain and obstructive jaundice.

IX-47. The answer is C. (Chap. 326; JW Newberger et al: Circulation 110:2747, 2004.) The most likely cause of the acute coronary syndrome in this patient is thrombosis of a coronary artery aneurysm in an individual with a past history of Kawasaki disease. Kawasaki disease is an acute multisystem disease that primarily presents in children less than 5 years of age. The clinical manifestations in childhood are nonsuppurative cervical lymphadenitis; desquamation of the fingertips; and erythema of the oral cavity, lips, and palms. Approximately 25% of cases are associated with coronary artery aneurysms that occur late in illness in the convalescent stage. Early treatment (within 7–10 days of onset) with IV immunoglobulin and high-dose aspirin decreases the risk of developing coronary aneurysms to about 5%. Even if coronary artery aneurysms develop, most regress over the course of the first year if the size is smaller than 6 mm. Aneurysms larger than 8 mm, however, are unlikely to regress. Complications of persistent coronary artery aneurysms include rupture, thrombosis and recanalization, and stenosis at the outflow area. Dissection of the aortic root and coronary ostia is a common cause of death in Marfan’s syndrome and can also be seen with aortitis due to Takayasu’s arteritis. In this patient, there is no history of hypertension, limb ischemia, or systemic symptoms that would suggest an active vasculitis. In addition, there are no other ischemic symptoms that would be expected in Takayasu’s arteritis. Myocardial bridging overlying a coronary artery is seen frequently at


autopsy but is an unusual cause of ischemia. The possibility of cocaine use as a cause of myocardial ischemia in a young individual must be considered, but given the clinical history it is a less likely cause of ischemia in this case.

IX-48. The answer is C. (Chap. 327) Recurrent oral ulceration is required for the diagnosis of Behçet’s disease. The ulcers may be single or multiple, are shallowly based with a yellow necrotic base, and are painful. They are generally small, less than 10 mm in diameter. The diagnosis of Behçet’s also requires two of the following: recurrent genital ulceration, eye lesions, skin lesions, and pathergy test. Nonspecific skin inflammatory reactivity to any scratches or intradermal saline injection (pathergy test) is common and specific.

IX-49 and IX-50. The answers are A and C, respectively. (Chap. 327) Behçet’s syndrome is a multisystem disorder of uncertain cause that is marked by oral and genital ulcerations and ocular involvement. This disorder affects males and females equally and is more common in persons of Mediterranean, Middle Eastern, and Far Eastern descent. Approximately 50% of these persons have circulating autoantibodies to human oral mucosa. The clinical features are quite varied. The presence of recurrent aphthous ulcerations is essential for the diagnosis. Most of these patients have primarily oral ulcerations, although genital ulcerations are more specific for the diagnosis. The ulcers are generally painful, can be shallow or deep, and last for 1–2 weeks. Other skin involvement may occur, including folliculitis, erythema nodosum, and vasculitis. Eye involvement is the most dreaded complication because it may progress rapidly to blindness. It often presents as panuveitis, iritis, retinal vessel occlusion, or optic neuritis. This patient also presents with superficial venous thrombosis. Superficial and deep venous thromboses are present in one-fourth of these patients. Neurologic involvement occurs in up to 10%. Laboratory findings are nonspecific with elevations in the erythrocyte sedimentation rate and the white blood cell count. Treatment varies with the extent of the disease. Patients with mucous membrane involvement alone may respond to topical steroids. In more serious or refractory cases, thalidomide is effective. Other options for mucocutaneous disease include colchicines and intralesional interferon α. Ophthalmologic or neurologic involvement requires systemic glucocorticoids and azathioprine or cyclosporine. Life span is usually normal unless neurologic disease is present. Ophthalmic disease frequently progresses to blindness.

IX-51. The answer is C. (Chap. 328) Relapsing polychondritis is a disease of unknown cause characterized by inflammation of the cartilage predominantly in the ears, nose, and laryngotracheobronchial tree. Although it may be a primary disorder, relapsing polychondritis is often associated with a number of other conditions including systemic vasculitis, systemic lupus erythematosus, Sjögren’s syndrome, spondyloarthritides, Behçet’s disease, inflammatory bowel disease, primary biliary cirrhosis, and myelodysplastic syndrome. It is not associated with scleroderma, which causes distinct skin changes that are typically not inflammatory and are not associated with cartilaginous inflammatory disease.

IX-52. The answer is E. (Chap. 328) Relapsing polychondritis most often presents with recurrent painful swelling of the ear. Although other cartilaginous sites may be involved such as the nose and the tracheobronchial tree, these are less frequent. Episodes of ear involvement may result in floppy ears. Typically the pinna is affected while the earlobe is spared, as there is no cartilage in the lobe. Cogan’s syndrome is a rare vasculitic syndrome involving hearing loss, but cartilage inflammation is not a feature. Recurrent trauma or irritation is a consideration but the history is not suggestive, and would be


less likely to be bilateral and accompanied by inflammatory findings and a relatively spared ear lobe.

IX-53. The answer is A. (Chap. 329) The finding of noncaseating granulomas is highly suggestive of sarcoidosis, but not confirmatory. To make a specific diagnosis, two or more organs must be affected; however, a suggestive radiograph and positive biopsy are often adequate. Prior to a definitive diagnosis, however, other conditions that may cause noncaseating granulomas should be ruled out. These include beryllium exposure, often in workers in the nuclear industry; atypical mycobacterial infection; and fungal infection such as histoplasmosis. Many malignancies, including testes, and a number of lymphomas may have granulomas, particularly in reactive areas near tumor deposits. Adequate tissue sampling must be ensured to rule out malignancy. Pulmonary alveolar proteinosis is a disease characterized by PAS-positive protein deposits in the alveolar spaces. It is not typically associated with acute or chronic inflammation in the absence of secondary infection.

IX-54. The answer is A. (Chap. 329) Hypercalcemia and/or hypercalciuria occurs in approximately 10% of patients with sarcoidosis and is thought to be due to increased production of 1, 25-dihydroxyvitamin D by the granuloma itself, with resultant increased gut calcium absorption. Sun and exogenous vitamin D can exacerbate the problem. Because of this, renal calculi are relatively common. If a patient with sarcoidosis is to begin therapy with calcium supplementation, 24-hour urine for calcium excretion should be performed before and after initiation of therapy. Often small doses of glucocorticoids are adequate to control this problem.

IX-55. The answer is A. (Chap. 329) The treatment of sarcoidosis depends on whether it is in the acute or chronic form. In many cases, acute disease without abnormalities of neurologic, cardiac, ocular, or metabolic systems may not require therapy. The mainstay of systemic therapy remains to be glucocorticoids. In the chronic form, treatment is dependent on the response to corticosteroids and the tolerability of tapering to low doses (<10 mg/d). Hydroxychloroquine has been shown to be effective in skin disease due to sarcoidosis. Methotrexate works in approximately two-thirds of patients, regardless of disease manifestations. Azathioprine is another cytotoxic agent that is often used in chronic disease, although the evidence in support of this therapy is mostly retrospective. Prospective studies of etanercept and infliximab in patients have shown that etanercept has a limited role as a steroid-sparing agent. In contrast, infliximab, when added to a regimen including prednisone and a cytotoxic agent, improved lung function. The difference in responses to these anti-TNF agents may have to do with the difference in mechanism (receptor antagonism vs. antibody).

IX-56. The answer is A. (Chap. 329) Sarcoidosis is often discovered on routine chest radiograph by the presence of bilateral hilar adenopathy, often in asymptomatic patients. The Scadding method of scoring the standard chest radiograph is still utilized despite CT being more sensitive. Stage 1 is hilar adenopathy (+/- right paratracheal adenopathy) alone; stage 2 is adenopathy plus parenchymal infiltrates; stage 3 is parenchymal infiltrates alone; and stage 4 is fibrosis. When parenchymal disease is present, the upper lobes often predominate in contrast to most other lung disorders. Skin involvement includes erythema nodosum, maculopapular lesions, hyper- and hypopigmentation, keloid formation, subcutaneous nodules, and lupus pernio. Erythema nodosum is usually seen in the acute form of sarcoidosis and often portends a positive prognosis. Liver involvement is difficult to assess. Granulomas may be seen in over 50% of liver biopsies in patients with sarcoidosis, but approximately 20% have evidence by laboratory studies. The typical pattern is an elevation of alkaline phosphatase


typical of all forms of granulomatous hepatitis. There may be some accompanying elevation of transaminases. Eye disease is most commonly anterior uveitis, although posterior (retinal and pars planitis) involvement may occur. There is a marked racial difference in eye involvement due to sarcoidosis. In Japan, over 70% of patients have eye disease, whereas in the United States the prevalence is about 30% (with it being more common in African Americans than white Americans). Cardiac involvement varies dramatically by race. In the United States and Europe, less than 5% of patients develop cardiac disease, whereas in Japan the prevalence is over 25%. There is no difference between whites and African Americans. Cardiac manifestations are conduction and systolic dysfunction due to granulomatous inflammation and infiltration.

IX-57. The answer is C. (Chap. 331) Inflammatory arthritis can have a variety of causes, either acute or chronic, and affect single or multiple joints. Acute causes of inflammatory arthritis most commonly include infectious etiologies (Neisseria gonorrhoeae, septic arthritis, Lyme disease) and crystal-induced arthropathies (gout, pseudogout). Chronic inflammatory arthritis is more likely to be related to autoimmune diseases such as rheumatoid arthritis, reactive arthritis, or psoriatic arthritis. A frequent feature of inflammatory arthritis is the presence of morning stiffness. The stiffness of inflammatory arthritis is more severe after a prolonged rest period, which is why it is characteristically worse in the morning. The stiffness can persist as long as an hour or more, is quite severe, and improves with movement. In contrast, noninflammatory arthritis such as osteoarthritis is more typically associated with stiffness that occurs after a brief rest, lasts less than 60 minutes, and worsens with increased activity. Individuals with inflammatory arthritis also frequently have associated systemic symptoms such as fatigue, fever, rash, or weight loss. On physical examination, one should observe the affected joints for signs of inflammation including redness, swelling, warmth, and pain with movement. Nonspecific laboratory evidence of inflammation is often present, including elevation of erythrocyte sedimentation rate, C-reactive protein, and platelet count, anemia of chronic disease or hypoalbuminemia.


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