A 55-year-old woman has had several weeks of fever, abdominal pain, weight loss, and lack of energy. Three days prior to the assessment, she developed a left foot drop. Her 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 antineutrophil cytoplasmic antibody (ANCA) and ANA. The eosinophil count is normal, and urinalysis is negative for casts, protein, and red cells. Which of the following is the most likely mechanism for renal injury in this condition?
This patient has polyarteritis nodosa (PAN) and in classic PAN, unlike microscopic polyangiitis, both small and medium vessels are involved. The renal lesions are ischemic secondary to fibrinoid necrosis of the vessels. In microscopic polyangiitis, a diffuse glomerulonephritis is frequently present. The most common organ systems involved are the kidneys, musculoskeletal system, and peripheral nervous system.
A 20-year-old man with Marfan syndrome is having back pain. On examination, there is a curvature of his thoracic spine to the left. Which of the following is the most appropriate next step in management?
The major musculoskeletal issue is progressive scoliosis, which is usually treated with physiotherapy and mechanical bracing. Only severe scoliosis is treated with surgery. Vigorous exercise and pregnancy are felt by some experts to increase the rate of aortic root dilatation.
A 27-year-old man has a history of low back pain and stiffness. Recently, he has noticed more severe stiffness at night and hip pain. The symptoms improve in the morning after doing some “stretching” exercises. On physical examination, there is paravertebral muscle and sacroiliac joint tenderness with limited flexion of the lumbar spine. A 2/6 diastolic murmur is also heard at the left sternal border radiating to the apex. Which of the following is the most likely diagnosis for the diastolic murmur?
The frequency of aortic insufficiency has been about 4% in ankylosing spondylitis (AS). Other cardiac valve anomalies are not increased in incidence. Rarely, congestive heart failure or third degree heart block can occur as well.
A 35-year-old woman notices a red rash over her cheeks, and pain and swelling in both knees as well as several small joints in her hands. She has a history of hypertension and 2 months ago was started on a new medication to control her blood pressure. Her ANA and antihistone antibodies are positive. Which of the following medications is most likely to cause her condition?
Hydralazine can cause drug-induced lupus (defined by positive ANA and antihistone antibodies). About 25–30 % of patients treated chronically with hydralazine will develop ANA positivity and about 10–20% of patients with ANA positivity will develop systemic symptoms compatible with lupus, particularly arthralgias. Genetic variation in drug acetylation rates might be a predisposing factor.
A 57-year-old man presents with a swollen, painful left knee. He fell on the knee 3 days ago while hurrying up the stairs. On examination, there is a swollen knee with palpable effusion and decreased range of motion. A diagnostic tap is preformed and 5 cc of transparent fluid is removed. The WBC count is 1000/mL (20% polymorphonuclear neutrophils [PMNs]), glucose is equal to plasma, viscosity is high, and lactate dehydrogenase (LDH) is low.
This man has a noninflammatory effusion likely from the trauma caused by the fall. In the noninflammatory category, the fluid is transparent, WBC 200–2000/mL (<25% PMNs), glucose is normal, and LDH is low. Another cause for a noninflammatory effusion is osteoarthritis.
A 77-year-old woman presents with a swollen, painful right knee. She fell on the knee 3 days ago while hurrying up the stairs. On examination, there is a swollen knee with palpable effusion and decreased range of motion. A diagnostic tap is preformed and 5 cc of opaque fluid is removed. The WBC count is 20,000/mL (50% PMNs), glucose is lower than plasma, viscosity is low, and the LDH is high.
This woman has an inflammatory category of effusion in view of the opaque color, high WBC 2000–10,000 (>50% PMNs), low glucose, and high LDH. Common causes for this include crystal induced arthritis, SLE, and RA. In septic arthritis, WBC is usually 50,000/mL or more and often >100,000/mL with >75% PMNs. Other important tests on synovial fluid include Gram stain and culture when an inflammatory effusion is suspected clinically.
A 27-year-old woman presents with a red rash over her cheeks, and pain and swelling in both knees as well as several small joints in her hands. She notes that the rash is worse with sun exposure. Medical evaluation reveals oral ulceration, positive ANA, and 3+ proteinuria. Which of the following is the most likely mechanism for the renal damage in this condition?
Renal disease is usually secondary to deposition of circulating immune complex. Although most patients with SLE have such deposits, only half have clinical nephritis as defined by proteinuria. Renal biopsy can provide both prognostic and therapeutic information.
A 50-year-old man develops fatigue and painful swelling of both hands. He is also very stiff in the morning and requires longer time to get ready for work. Physical examination reveals erythema, swelling, and tenderness on palpation of the proximal interphalangeal joints and MCP joints. Plain x-rays of the hand are taken. Which of the following x-ray findings is characteristic of this condition?
This patient has features of rheumatoid arthritis, and early in RA there may not be any bony changes seen, except nonspecific findings of soft tissue swelling and joint effusions. With longer active inflammation of the joints, loss of cartilage and bony erosions can be seen. The value of x-rays is to determine the extent of bone and cartilage damage.
A 23-year-old man notices new low back pain, stiffness, and left eye discomfort. Sunlight also bothers his eyes. The back pain is worse at night and described as a dull ache in the back and buttock area. On physical examination, there is paravertebral muscle, iliac crest, and ischial tuberosity tenderness with limited flexion of the lumbar spine. His eye is inflamed and the pupil is constricted. Pelvic x-rays show sacroiliitis. Which of the following is the most likely diagnosis for his eye symptoms (it is the most common extra-articular manifestation of this condition)?
Acute anterior uveitis is the most common extra-articular manifestation of ankylosing spondylitis (AS). Pain, photophobia, and increased lacrimation are the usual symptoms. Attacks are unilateral and tend to recur, often in the other eye. Cataracts and secondary glaucoma are not uncommon sequelae. The iritis is usually managed with local glucocorticoid administration in association with a mydriatic agent.
A 24-year-old man presents with shortness of breath on exertion. He is tall and thin (Fig. 9–2), visual acuity is poor with his glasses off, the jugular venous pressure (JVP) is 4 cm, first heart sound is normal, second sound is soft, a 3/6 early diastolic murmur is heard at the right second intercostal space radiating to the apex. The pulses are bounding, lungs are clear, and his arms are quite long in comparison to his total length. Which of the following is the most likely diagnosis for his cardiac murmur?
The patient has Marfan syndrome (triad of long thin extremities, lens dislocation, and aortic aneurysms). Aortic involvement occurs in about 80%, with degenerative changes predominating. Aortic root dilatation can cause aortic regurgitation or aortic aneurysm and rupture.
A 40-year-old woman complains of being very “stiff” in the morning, in addition to having sore hands and wrists. The symptoms have come on very gradually and she can not recall exactly when they started. Her only other complaint is that of being fatigued. Physical examination reveals involvement of the proximal interphalangeal joints, MCP joints, and wrist joints in a symmetrical fashion. She also has nodules over both elbows. Which of the following is predictive of developing extraarticular features of her condition?
Extra-articular manifestations of RA generally develop in patients with high titers of autoantibody to the Fc component of IgG (also known as rheumatoid factor (RF)
A 24-year-old is referred for assessment of a low white cell count. She has no past medical history and is not on any medications. Her only symptoms are of joint discomfort in her hands, and occasional sharp chest pains that change with breathing. On examination, there is inflammation of some MCP and DIP joints in both hands, and the rest of the examination is normal. Her WBC is 3500/mL and the lymphocytes are low (15%) and PMNs are normal. Which of the following is the most likely diagnosis?
Leukopenia occurs in almost two-thirds of the SLE patients, and the differential count is usually normal. Lymphocytes and platelets can also be reduced.
A 69-year-old man develops clubbing of his fingers. Which of the following is most likely associated with this condition?
Mononuclear cell infiltration and edema develop in the periosteum, synovial membrane, and joint capsule. Secondary hypertrophic osteoarthropathy (HOA) (e.g., lung cancer) is more common than primary HOA.
A 30-year-old woman develops painful swelling of her hands, pleuritic chest pain, and shortness of breath on exertion. She is also very stiff in the morning. Physical examination reveals involvement of the proximal interphalangeal joints and MCP joints. Air entry is decreased to the right lower lobe and the area is dull on percussion. Her RF is elevated and a CXR reveals a pleural effusion. A diagnostic tap is performed, and 500 cc of a straw-colored fluid is removed. Which of the following biochemical patterns is consistent with a pleural effusion due to her primary condition?
Pleuritis is common at autopsy in patients with RA but is not usually symptomatic. Typically, the pleural fluid shows increased pleural fluid: serum protein and LDH ratios (exudate pattern), and low glucose and low complement levels. Pleuropulmonary manifestations are more common in men with RA.
A 67-year-old man has a long history of symmetrical small joint arthritis with deformities. He now develops shortness of breath on exertion with a dry cough, but no sputum or chest discomfort. His heart sounds have a loud P 2 , and the lungs have fine bibasilar crackles. Which of the following is the most likely diagnosis of the pulmonary condition associated with his arthritis?
In RA, pleural involvement is very common at autopsy but infrequently causes symptoms. Interstitial lung disease (ILD) is the most common manifestation of rheumatoid lung disease. RA associated interstitial lung disease (RA-ILD) is usually similar to idiopathic pulmonary fibrosis (IPF) in terms of its clinical presentation, pathology, disease spectrum, and pathogenesis. Presentation is more common at age 50–60 years, in men (M:F = 2–3:1), and in association with seropositive and erosive joint disease. If pleural fluid is present, glucose levels are very low.
A 71-year-old woman comes to the office with a history of headaches, fatigue, and weight loss for 3 months. The headaches are new for her, and usually not very severe. Her jaw also hurts when she is chewing food. Two days prior, she had briefly lost partial vision in her left eye. There were no other neurologic symptoms at the time. On examination, her neck is supple to flexion, fundi and neurologic examinations are normal. She is started on prednisone 60 mg/day and a biopsy is performed to confirm the diagnosis. Which of the following is the most likely change seen on the biopsy to confirm the diagnosis?
Temporal artery biopsy is required for definitive diagnosis of giant cell arteritis, because of the relatively nonspecific nature of the presenting symptoms, signs, and routine laboratory tests. The arteritis can be segmental, however, and great care must be taken in the pathologic assessment.
A 30-year-old woman develops a rash over her cheeks, nose, and ears. She also has pain and swelling in her wrists as well as several small joints in her hands. Medical evaluation reveals oral ulceration and 3+ proteinuria. Her ANA is positive. Which of the following is the most likely cardiac manifestation of her disease?
This patient has SLE and pericarditis, sometimes leading to tamponade, is the most common manifestation of cardiac disease. Myocarditis does occur and can cause arrhythmias, sudden death, or heart failure. Libman-Sacks endocarditis is associated with thrombotic events or, less commonly, valvular regurgitation. Myocardial infarction is more commonly a result of atherosclerotic disease than vasculitis.
A 32-year-old woman has a long history of intermittent bloody diarrhea and crampy abdominal pain. Recently, her bowel symptoms have increased and she is having 4–6 bowel movements a day with mucus and blood in the stool. She now has a low-grade fever, loss of appetite, and new musculoskeletal symptoms. Which of the following is the best description of this patient’s accompanying arthritis?
In inflammatory bowel disease, there are two common types of arthritis involvement. The first is an asymmetric, migratory polyarthritis that affects the large joints of the lower and upper extremities and is closely related to the activity of bowel disease. Spondylitis is also common (though not always symptomatic) and is not always related to activity of bowel disease.
A 42-year-old woman has had several weeks of fever, abdominal pain, weight loss, and lack of energy. Three days prior to assessment, she developed a left foot drop and rash on her legs. Her 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. The rash looks like livedo reticularis. 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, and urinalysis is negative for casts, protein, and red cells. Biopsy of the skin rash shows inflammation of the small blood vessels. Which of the following is the most appropriate next step in management?
Current treatment for polyarteritis nodosa (PAN) mimics that of Wegener’s granulomatosis in the initial treatment with combination steroid and cyclophosphamide therapy. This will result in up to a 90% long-term remission rate even after discontinuation of therapy. In cases associated with hepatitis B infection, plasmapheresis is sometimes used as initial therapy.
A 67-year-old man complains of frequent headaches that are new for him. They are usually not very severe, and relieved with acetaminophen. He also has some back, shoulder, and hip discomfort, which is worse in the morning as well he feels quite fatigued and does not have his usual energy level. On examination, his neck is supple to flexion, fundi and thyroid examination are normal. Range of motion in the shoulders and hips is reduced because of discomfort but there is no active inflammation. There are no focal deficits on screening neurologic examination. Which of the following is the most appropriate initial diagnostic test?
Almost all patients with temporal arteritis will have an elevated ESR. Although a high ESR cannot make the diagnosis, a normal ESR helps in excluding the diagnosis. C-ANCA is a diagnostic tool for Wegener’s granulomatosis. Elevated CPK is not seen in temporal arteritis, even with associated PMR. Normochromic, or slightly hypochromic, anemia often seen in temporal arteritis is too nonspecific to be of much diagnostic help.
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