A 45-year-old man has had several weeks of fever, abdominal pain, weight loss, and lack of energy. Three days prior to assessment, he developed a left foot drop. His blood pressure is 160/90 mm Hg, pulse 80/min, and physical examination confirms left peroneal nerve damage and a bilateral sensory peripheral neuropathy in both legs. There are no skin rashes. Laboratory evaluation reveals ESR of 105 mm/h, WBC of 14,000/mL, and negative serologic tests for ANCA and ANA. Eosinophil count is normal. Which of the following chronic viral infections is sometimes associated with this condition?
About 20–30% of patients with PAN have hepatitis B antigenemia. Circulating immune complexes containing hepatitis B antigen and immunoglobulin have been detected, and immunofluorescence of blood vessel walls have also demonstrated hepatitis B antigen. Antiviral therapy has been used in these cases.
A 19-year-old man has a chronic papulosquamous skin disorder involving his knees and elbows. He now has dull low back pain and morning stiffness that lasts a few hours. On examination, forward flexion at the lumbar spine is reduced but neck movements are normal. There is stress tenderness of both sacroiliac joints. Which of the following is the most likely diagnosis?
This patient has a sacroiliac form of arthritis seen in patients with psoriasis. Patients with psoriasis can develop five different patterns of musculoskeletal symptoms. These include arthritis of the DIP joints; asymmetric oligoarthritis; symmetric polyarthritis similar to RA; spine and sacroiliac type; and arthritis mutilans, a highly destructive form. Most patients with psoriatic arthritis also have nail involvement. Only about a quarter actually develop a progressive, destructive disease. Uric acid may be elevated because of high tissue turnover but is not part of the pathogenesis of joint disease.
A 63-year-old man is worried that he has RA because his RF serology is positive in a low titer. He has pain in his left hand and right knee, which usually bother him in the evening. He has not noticed any inflammation or swelling and there is no history of morning stiffness. On examination, there are no active joints. Which of the following statements regarding the presence of RF is correct?
The presence of RF has little predictive power in determining the diagnosis of RA. However, it is useful in determining prognosis, as high titers of RF are associated with more severe and progressive disease, as well as with extra-articular manifestations.
A 45-year-old man has had intermittent swelling and pain in the superior part of his auricles for several years. Mild arthritis usually accompanies these episodes. Last year he also had redness, pain, and swelling over the bridge of his nose. Which of the following is the most likely diagnosis?
The disease is relapsing polychondritis and is characterized by frequent remissions and exacerbations of lesions and is rarely fatal. Auricular chondritis and nasal chondritis are the most common manifestations. It can also be secondary to SLE, RA, Sjögren’s syndrome, and vasculitis.
Which of the following features is a characteristic of Ehlers-Danlos syndrome?
In Ehlers-Danlos syndrome, skin hyperextensibility, fragility, and bruisability are marked, and this condition may create difficulties at operation. Habitual dislocation of joints is also a characteristic of this syndrome.
A 72-year-old man injures his right knee in a car accident, and now it is swollen and extremely painful to bend the knee. X-rays of the knee rule out a fracture, and joint fluid aspiration reveals an opaque-colored fluid containing rhomboid crystals with weak-positive birefringence. Which of the following is the most appropriate next step in management?
In traumatic arthritis, swellings, ecchymoses, muscular spasms, and tenderness tend to be present, but fractures must be excluded. This man has calcium pyrophosphate (CPPD) crystalinduced monoarthritis, so called “pseudogout.” It is most common in the elderly and can be precipitated by minor trauma. The crystals have a rhomboid shape, and the clinical presentation can mimic that of gout. It can be associated with metabolic abnormalities such as hyperparathyroidism or hemochromatosis. Treatment is with an NSAID for 7–10 days. If there are multiple joints involved, then steroids can be considered. An alternative to oral NSAIDs is intra-articular steroids for single joint disease. Allopurinol is not effective in CPPD. Synovial fluid is bloody, but the fluid is of normal viscosity, so a “string” test is usually positive.
A 64-year-old man is having difficulty getting out of a chair. He has lost 15 lb and feels tired all the time. On examination, there is a blue purple rash on his eyelids and knuckles, and muscle strength in his proximal muscles is rated 4 out of 5. His creatinine kinase (CK) level is elevated and he is started on prednisone. Which of the following is the most important in monitoring response to therapy?
The course of muscle necrosis in dermatomyositis can be best followed by repeated CK determinations. Repeated muscle biopsies are rarely required. However, the goal of therapy is to increase muscle strength and function, so following muscle strength is the key clinical assessment of response to therapy.
A 25-year-old woman with SLE notices increasing fatigue and shortness of breath on exertion. Her usual SLE symptoms of joint discomfort, chest pain, and fevers are not present. On examination, there are no active joints, but she is jaundiced. Laboratory studies show hemoglobin 9 g/dL, WBC 5000/mL, platelets 150,000/mL, reticulocyte count 4%, direct antiglobulin test: positive, total bilirubin 4 g/dL, direct 0.5 mg/dL, aspartate amino transferase (AST) 20 U/L, alanine amino transferase (ALT) 15 U/L, LDH 300 U/L. Which of the following is the most likely diagnosis?
This patient with SLE has developed autoimmune hemolytic anemia. Treatment consists of high-dose oral steroids. The positive Coombs’ test (direct antiglobulin test), high indirect bilirubin, high LDH, and high retic count are all supportive of hemolytic anemia. A normal AST and ALT rule out hepatitis.
A 38-year-old man has had 3 months of intermittent joint pain and swelling in both knees. The symptoms last weeks at a time and then improve spontaneously. His only past medical history is that of an oval-shaped, red pruritic skin lesion in his right axilla 4 months earlier, which appeared shortly after a camping trip. His immunoglobulin G (IgG) serology for Borrelia burgdorferiis positive. Which of the following is the most appropriate next step in management?
This story is typical of Lyme disease. The spirochete involved (B. burgdorferi) is transmitted by ixodic ticks and is most common in the Northeastern and Midwestern parts of the United States. The host animal varies depending on the exact type of tick. Treatment is briefer and more effective earlier in the course of the disease. The treatment of choice is doxycycline 100 mg bid for 1–2 months. Amoxicillin is a second choice drug.
A 26-year-old man has had a year of intermittent joint pain and swelling in both knees. The symptoms last weeks at a time and then improve. He has always been healthy. His only past medical history is that of a round, pruritic skin lesion in his left groin 2 years earlier, which appeared shortly after a camping trip. Which of the following is the most appropriate initial diagnostic test?
This story is typical of Lyme disease. The spirochete involved (B. burgdorferi) is transmitted by ixodic ticks and is most common in the Northeastern and Midwestern parts of the United States. The host animal varies depending on the exact type of tick. Because antibody studies cannot differentiate between active and inactive disease, the appropriate constellation of symptoms is also required for diagnosis.
Which of the following statements concerning the articular manifestations of RA is correct?
As in most inflammatory arthritides, the patient with RA generally has morning stiffness for more than 1 hour. Wrist involvement is nearly universal and is associated with radial deviation (unlike the ulnar deviation of the digits) and carpal tunnel syndrome. Hand involvement characteristically involves the proximal interphalangeal and MCP joints in a symmetric involvement. High fever (>100.4°F), even with active synovitis, should suggest an intercurrent problem such as infection.
A 22-year-old man has symptoms of low back pain and stiffness. After several months of mild symptoms, he notes more severe stiffness at night and hip pain. On physical examination, there is paravertebral muscle tenderness and limited flexion of the lumbar spine. Figure 9–3 shows an x-ray of the lumbar spine. Which of the following is the most likely diagnosis?
AS occurs in 1–6% of adults inheriting human lymphocyte antigen B27 (HLA-B27). However, the prevalence in B27-positive relatives of patients with AS is up to 30%. Men are three times more likely to be affected.
A 64-year-old woman presents with fatigue, and musculoskeletal symptoms. She reports of no headache, jaw discomfort, or visual disturbance. Her physical examination is normal, and laboratory testing reveals an elevated ESR of 75 mm/h. She is started on prednisone 10 mg/day and notices a dramatic improvement in her symptoms after 1 week. Which of the following are the most typical symptoms of this disorder?
This patient has PMR. It is characterized by stiffness, aching, and pain in proximal muscle groups in the neck, shoulders, back, hips, and thighs. It is considerably more common than temporal arteritis. Both diseases are almost exclusively seen in the over 50 age group.
A 69-year-old man comes to the office complaining of fatigue, and weight loss for 3 months. He also reports having frequent headaches, which are new for him. There are no other constitutional symptoms of fever, chills or night sweats. He does have chronic lower back pain but lately he has noticed pain in his shoulder, and hip as well. The muscle and joint symptoms are worse in the morning, and the stiffness lasts for 1 hour. His jaw also hurts when he is chewing food. Head and neck examination is normal, there is no lymphadenopathy and fundoscopy is normal. Range of motion in the shoulders and hips is reduced because of discomfort but there is no active inflammation. Which of the following is the most feared complication in patients with this condition?
Although all these complications have been reported in giant cell arteritis, the only one with a significant likelihood is blindness secondary to ischemic optic neuropathy. Thus, if the disease is suspected, urgent diagnosis and treatment is required.
A 67-year-old woman has pain in her left hand and right knee, which is interfering with her activities. The pain came on gradually, first in her hand 6 months ago and now in her knee. It is usually fine when she wakes up, but gets worse as the day progresses. There is no history of any trauma, and she is otherwise well. Taking over-the-counter NSAIDs usually relieves the pain. On examination, there is bony soft tissue swelling of his second and third DIP joints in the left hand and crepitus over the right knee with flexion. There is no erythema or joint effusion. Which of the following best describes this disease condition?
Although the hallmark of osteoarthritis is the progressive loss of articular cartilage, it is best considered as a disease of the entire organ, the synovial joint, rather than of any of its component tissues. In fact, all areas of the joint, bone, cartilage, synovium, meniscus, and ligaments are involved.
A very tall, slender 16-year-old boy is referred for evaluation. Physical examination reveals long fingers, pectus excavatum, and a high arched palate. Which of the following is most likely to be seen on his CXR?
In Marfan syndrome, inheritance is autosomal dominant, and the aortic lesion is a cystic medial necrosis with loss of elastic tissue, resulting in aneurysm formation. Pneumothorax can occur but is not as characteristic. Mitral valve prolapse can also be part of the syndrome. Dislocation of the lens is the most apparent eye abnormality. Severe chest deformities and long limbs are characteristic. High, arched palate; high pedal arches; and pes planus are common.
A 29-year-old woman develops symptoms of painful swelling, and stiffness of both hands. Physical examination reveals involvement of the proximal interphalangeal joints and MCP joints. A clinical diagnosis of rheumatoid arthritis is made. Which of the following is the most likely cause of the inflammation in her joints?
Numerous mediators of inflammation are found in the synovium of patients with rheumatoid arthritis (RA). The evidence favoring activated T cells as the initiators of the inflammation include the predominance of CD4+ T cells in the synovium, the increase in soluble interleukin-2 (IL-2) receptors (a product of T-cell activation), and amelioration of symptoms by T-cell removal.
A 25-year-old woman develops painful swelling of both hands and wrists. She is also very stiff in the morning. Physical examination reveals erythema, swelling and joint line tenderness of the proximal interphalangeal, MCP, and wrists joints. Her RF is positive, ANA is negative, and x-rays of the hands show early erosive joint changes. Which of the following medications is most likely to prevent progression of disease?
Methotrexate, 7.5–20 mg once weekly, is the most commonly recommended disease modifying drug, because its effect is more rapid and patients are able to tolerate it for longer periods of time. Maximum improvement with methotrexate occurs after 6 months of therapy. Toxicity includes GI upset, oral ulceration, and liver function abnormalities. GI upset in particular may be ameliorated by concurrent folic acid administration. Pneumonitis has also been reported.
A 22-year-old woman develops a red rash over her cheeks, and pain and swelling in both knees as well as several small joints in her hands. Medical evaluation reveals oral ulceration and 3+ proteinuria. Which of the following is the most sensitive test for the diagnosis of this condition?
ANAs are present in 98% of patients with SLE. Repeatedly negative tests make the diagnosis of SLE very unlikely. Unfortunately, the test is not specific and may be positive in normal people (especially in older individuals), or secondary to infections, drugs, or other autoimmune disorders.
A 39-year-old man has had several weeks of fever, abdominal pain, weight loss, and lack of energy. Three days prior to assessment, he developed a left foot drop. Physical examination confirms left peroneal nerve damage and a bilateral sensory peripheral neuropathy in both legs. Laboratory evaluation reveals ESR of 105 mm/h, WBC of 14,000/mL, and a negative serologic test for ANCA. The eosinophil count is normal. Which of the following is a reasonable method of establishing a diagnosis?
This patient likely has PAN. ANCA and other serology are usually negative in PAN; positive serology suggests another diagnosis. The optimal diagnostic strategy is the biopsy of an affected organ. However angiography to look for aneurysms of small- and mediumsized arteries generally has higher yield than blind biopsy of unaffected organs. The lungs are not a characteristic site of involvement.
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