A 45-year-old woman has pain in her fingers on exposure to cold, arthralgias, and difficulty swallowing solid food. She has a few telangiectasias over the chest but no erythema of the face or extensor surfaces. There is slight thickening of the skin over the hands, arms, and torso. What is the best diagnostic test?
The symptoms of Raynaud phenomenon, arthralgia, and dysphagia point toward the diagnosis of scleroderma. Scleroderma, or systemic sclerosis, is characterized by a systemic vasculopathy of small- and medium-sized vessels, excessive collagen deposition in tissues, and an abnormal immune system. It is an uncommon multisystem disease affecting women more often than men. There are two variants of scleroderma—a limited type (previously known as CREST syndrome) and a more severe, diffuse disease. Antinuclear antibodies are almost universal. Topoisomerase-I antibody occurs in only 30% of patients with diffuse disease, but a positive test is highly specific. Anti-centromere antibodies are more often positive in limited disease. Cardiac involvement may occur, and an ECG could show heart block but is not at all specific. Renal failure can develop insidiously, but BUN and creatinine levels would not be diagnostically specific. Rheumatoid factor is nonspecific and present in 20% of patients with scleroderma. Reproduction of Raynaud phenomena is nonspecific and is not recommended as an office test.
A 20-year-old man complains of arthritis and eye irritation. He has a history of burning on urination. On examination, there is a joint effusion of the right knee and a rash of the glans penis. Which of the following is correct?
Reactive arthritis (Reiter syndrome) is a reactive polyarthritis that develops several weeks after an infection such as nongonococcal urethritis (NGU) or gastrointestinal infection caused by Yersinia enterocolitica, Campylobacter jejuni, or Salmonella or Shigella species. Reiter syndrome is characterized as a triad of oligoarticular arthritis, conjunctivitis, and urethritis. The disease is most common among young men and is associated with the histocompatibility antigen, HLA-B27. Circinate balanitis is a painless red rash on the glans penis that occurs in 25% to 40% of patients. Other clinical features may include keratodermia blennorrhagicum (a rash on the palms and soles indistinguishable from papular psoriasis) and spondylitis. ANA and rheumatoid factor are usually negative. CPK would be elevated in polymyositis or dermatomyositis but not in reactive arthritis. Gonorrhea rarely precipitates Reiter syndrome, and a negative urethral culture would be expected.
A 22-year-old man presents to your office with complaint of right knee pain and swelling for the past 2 weeks. Although uncomfortable, it has not prevented ambulation. He denies any fevers or night sweats. He reports that he has had several similar episodes of knee and ankle pain over the past few months. He reports malaise and fatigue over the past few months as well. He has had four sexual partners over the past 6 months but denies dysuria or urethral discharge. With prompting, the patient recalls a rash on his arm accompanied by fever after a trip to upstate New York to attend a music festival. He felt the rash was interesting, and took a picture which he saved to his smart-phone (see below). Which of the following would be the most appropriate next step in the management of this disease?
The patient in question has Lyme disease. Lyme disease is caused by infection with the spirochete Borrelia burgdorferi, transmitted by the bite of the Ixodes tick. In the United States, most cases are reported in the Northeast and North-central parts of the country. The majority of patients develop a characteristic rash (erythema migrans) which slowly expands over days before resolving. As the spirochete disseminates, patients can develop aseptic meningitis, nerve palsies, and cardiac conduction abnormalities. Patients will frequently develop a migratory monoarticular arthritis that can recur intermittently for months to years. The diagnosis of Lyme disease is difficult if erythema migrans (EM) is not present. With the documentation of EM and a history consistent with Lyme disease (high suspicion) additional testing is not indicated, and the patient should be treated empirically with antibiotics. Answer c is incorrect because of the high pretest probability that this patient has Lyme disease. In the absence of an EM lesion, the diagnostic picture becomes murkier. For moderate pretest probability patients, it has been recommended that Lyme disease titers be tested. For low-probability patients, neither titers nor empiric therapy are recommended. Although the patient may benefit from STD testing and treatment after his attendance to the music festival (answer a), the rash shown is not consistent with chlamydial induced reactive arthritis. As the pathogenesis of Lyme disease is fundamentally one of infection rather than immune dysregulation, empiric steroid therapy would not be indicated (answer d). It would be highly unusual for rheumatoid arthritis (answer e) to present as primarily knee involvement in this demographic, and RA would not account for the associated rash.
A 75-year-old man complains of headache. On one occasion he transiently lost vision in his right eye. He also complains of aching in the shoulders and neck. There are no focal neurologic findings. Carotid pulses are normal without bruits. Laboratory data show a mild anemia. Erythrocyte sedimentation rate (ESR) is 85. Which of the following is the best approach to management?
Headache and transient unilateral visual loss (amaurosis fugax) in this elderly patient with polymyalgia rheumatica (PMR) symptoms suggest a diagnosis of temporal arteritis. The erythrocyte sedimentation rate is high in almost all cases. Temporal arteritis occurs most commonly in patients older than 55 and is highly associated with polymyalgia rheumatica. However, only about 25% of patients with PMR have giant cell arteritis. Older patients who complain of diffuse myalgias and joint stiffness, particularly of the shoulders and hips, should be evaluated for PMR with an ESR. Unilateral visual changes or even permanent visual loss may occur abruptly in patients with temporal arteritis. Biopsy results should not delay initiation of corticosteroid therapy. Biopsies may show vasculitis even after 14 days of glucocorticoid therapy. Delay risks permanent loss of sight. Once an episode of loss of vision occurs, workup must proceed as quickly as possible. Treatment for temporal arteritis requires relatively high doses of steroids, beginning with prednisone at 40 to 60 mg per day for about 1 month with subsequent tapering. Aspirin should be added because it decreases the risks of vascular occlusions but is not sufficient alone. The treatment for polymyalgia rheumatica without concomitant temporal arteritis requires much lower doses of steroids, in the range of 10 to 20 mg per day of prednisone. Carotid disease can cause amaurosis fugax but would not account for the headache, polymyalgia rheumatica, or the elevated sedimentation rate.
A 53-year-old woman presents with pain in the fingers bilaterally. Examination reveals inflammation of the synovium of multiple DIP and PIP joints. Larger joints are spared. Skin examination reveals the following lesion (pic).
What is the most likely diagnosis?
The patient has psoriatic arthritis. Psoriatic arthritis is an immune-mediated arthritis affecting up to 30% of patients with psoriasis. Although psoriasis usually precedes the development of arthritis, occasionally the joint symptoms come first. Classic manifestations include involvement of the DIP joints, presence of dactylitis (“sausage digit”), and of course the presence of psoriasis of the skin. Joint involvement patterns are variable, however, and can mimic rheumatoid arthritis (as in this case) or involve primarily the axial skeleton. Nail changes, including pitting, horizontal ridging, onycholysis, dystrophic hyperkeratosis, and yellowish discoloration are common. The diagnosis is primarily clinical, although radiographic evidence of “pencil in cup” deformity of the DIP joint may lend additional weight to the diagnosis. Immunosuppression is required to prevent deformation of the joints, with anti-TNF alpha agents being the current drug of choice. Although hemochromatosis can cause joint involvement, it typically mimics the noninflammatory presentation of osteoarthritis, often with involvement of the second and third MCP joints (an unusual pattern in primary osteoarthritis). The cardinal skin manifestation of hemochromatosis is diffuse hyper-pigmentation, leading to the colloquial description of “bronze diabetes.” Rheumatoid arthritis could cause the joint manifestations described here, but would not result in the portrayed skin lesion. RA classically affects the metacarpophalangeal (MCP) and spares the DIP joints. Osteoarthritis will not present with polyarticular synovitis or a psoriatic plaque. SLE can mimic many diseases, but discoid lupus lesions typically have an atrophic center with erythematous scaly edge, as opposed to the uniformly thick scale seen in psoriasis.
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