QUESTION: 181

A 55-year-old woman presents to your office with painful hands, causing difficulty opening jars and turning the key in the ignition of her car. She is fatigued and she notices joint stiffness, but limbers up by lunch. She has trouble getting her rings off because of enlarging knuckles. About a year ago, she tried some OTC ibuprofen, which seemed to help, but led to the development of a bleeding ulcer severe enough to require transfusion and ICU care. Otherwise, her health is good, and her review of systems is negative. Your physical examination reveals tenderness and swelling at the index proximal interphalangeal and metacarpophalangeal joints bilaterally. There are small effusions on both knees. She has tenderness to lateral compression of the forefoot area bilaterally.

The following data are obtained: normal CBC; normal basic metabolic panel; ESR 40 mm/h; ALT 90 U/L; AST 110 U/L; alkaline phosphatase 70 U/L; bilirubin 0.2 mg/dL; uric acid 5.1 mg/dL; urinalysis is normal. ACE level is normal. Rheumatoid factor is 60 and ANA is positive 1:40 speckled pattern. The next most important test would be which of the following?

hepatitis C antibody
anti-double-stranded DNA antibodies
serum protein electrophoresis
C-reactive protein
RPR

Answer(s): A

Explanation:

In all likelihood, this patient is presenting with a systemic inflammatory arthritis. Clearly, treatment will need to be initiated. In order to effectively and promptly treat her, you will need to understand the current state of her physiology. Therefore, basic laboratory studies including blood count, full chemistries, and urinalysis should be obtained. At this point, the most likely diagnosis is RA, and the rheumatoid factor and sedimentation rate may be helpful. Theoretically, sarcoidosis can present in this way but, epidemiologically, this is much less likely. Because of this and because the ACE level is fairly nonspecific, it should not be part of the initial workup. Neither joint fluid aspiration nor uric acid levels are likely to be diagnostic. The elevation of serum transaminase in the face of elevated sedimentation rate, moderate or low positive ANA, and rheumatoid factors raise the question about hepatitis C. About 50% of patients with active hepatitis C will have cryoglobulinemia. Cryoglobulins can produce low moderate positive rheumatoid factors. Therefore, it is extremely important in this circumstance to be certain that hepatitis C is not present. With such a low positive ANA, the likelihood that this is classical Lupus is low, and double-stranded DNA antibodies are not likely to be revealing. C-reactive protein may confirm the presence of inflammation, but it won't provide additional information over the sedimentation rate. Syphilis, "the great imitator," again may occasionally have arthritis as a manifestation--but rarely without other features. The remaining studies while they might be useful later but are unlikely to be helpful as the next most important test obtained. The probable source of the patient's symptoms is RA. Osteoarthritis can produce articular swelling, but on physical examination, there is rarely bogginess in the synovium. Anti-CCP antibody is an antibody directed against the citrullinated portion of fillagen. It has the highest specificity for RA of any antibody known. It is usually present early and may predict more severe disease.

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QUESTION: 182

A 55-year-old woman presents to your office with painful hands, causing difficulty opening jars and turning the key in the ignition of her car. She is fatigued and she notices joint stiffness, but limbers up by lunch. She has trouble getting her rings off because of enlarging knuckles. About a year ago, she tried some OTC ibuprofen, which seemed to help, but led to the development of a bleeding ulcer severe enough to require transfusion and ICU care. Otherwise, her health is good, and her review of systems is negative. Your physical examination reveals tenderness and swelling at the index proximal interphalangeal and metacarpophalangeal joints bilaterally. There are small effusions on both knees. She has tenderness to lateral compression of the forefoot area bilaterally.

The test ordered above is negative and an anticyclic citrullinated peptide (anti-CCP) antibody is strongly positive (600). Which of the following is the probable source of her symptoms?

cryoglobulinemia
osteoarthritis
polymyalgia rheumatica
SLE
RA

Answer(s): E

Explanation:

In all likelihood, this patient is presenting with a systemic inflammatory arthritis. Clearly, treatment will need to be initiated. In order to effectively and promptly treat her, you will need to understand the current state of her physiology. Therefore, basic laboratory studies including blood count, full chemistries, and urinalysis should be obtained. At this point, the most likely diagnosis is RA, and the rheumatoid factor and sedimentation rate may be helpful. Theoretically, sarcoidosis can present in this way but, epidemiologically, this is much less likely. Because of this and because the ACE level is fairly nonspecific, it should not be part of the initial workup. Neither joint fluid aspiration nor uric acid levels are likely to be diagnostic. The elevation of serum transaminase in the face of elevated sedimentation rate, moderate or low positive ANA, and rheumatoid factors raise the question about hepatitis C.

About 50% of patients with active hepatitis C will have cryoglobulinemia. Cryoglobulins can produce low moderate positive rheumatoid factors. Therefore, it is extremely important in this circumstance to be certain that hepatitis C is not present. With such a low positive ANA, the likelihood that this is classical Lupus is low, and double-stranded DNA antibodies are not likely to be revealing. C-reactive protein may confirm the presence of inflammation, but it won't provide additional information over the sedimentation rate. Syphilis, "the great imitator," again may occasionally have arthritis as a manifestation--but rarely without other features. The remaining studies while they might be useful later but are unlikely to be helpful as the next most important test obtained. The probable source of the patient's symptoms is RA. Osteoarthritis can produce articular swelling, but on physical examination, there is rarely bogginess in the synovium. Anti- CCP antibody is an antibody directed against the citrullinated portion of fillagen. It has the highest specificity for RA of any antibody known. It is usually present early and may predict more severe disease.

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QUESTION: 183

A 50-year-old man presents to your office with fatigue and weakness. He first noticed it a few weeks ago while trying to hang pictures with his wife. His legs have begun to ache as he walks up stairs. He has lost about 20 lbs in the last 3 months. Most recently, he has found that he is more constipated and has trouble rising from the commode. Your physical examination reveals modest proximal weakness, no articular swelling, rash, or any other pertinent findings. Blood work from a recent insurance examination revealed:
Sodium 142 meq/L; potassium 3.8 meq/L; chloride
107 meq/L; bicarbonate 29 meq/L; BUN
30 mg/dL; Cr 1.6 mg/dL; WBC 6.8; Hgb 13.6 g/dL;
HCT 40%; MCV 88.0 m3; platelets 240,000/mm3;
AST 200 U/L; ALT 250 U/L; alkaline phosphatase 70 U/L; bilirubin 0.3 mg/dL; ESR 40 mm/h. Along with a creatine phosphokinase (CPK), which of the following tests should be ordered first?

muscle biopsy
gamma glutamyl transferase (GGT)
MRI of the lumbar spine
ultrasound of the liver and gallbladder
kidney ultrasound with renal artery Doppler

Answer(s): B

Explanation:

The clinical features presented by the patient suggest a myopathy. It is often forgotten that serum transaminases are found in the muscle as well as the liver. Thus, a significant inflammatory myopathy may present with elevated serum transaminases in addition to symptoms. In this patient with normal alkaline phosphatase and bilirubin, initial measurement of the GGT would help rule out liver pathology and would be more appropriate initially than ultrasonography. Rhabdomyolysis may lead to renal dysfunction or even renal failure and a kidney ultrasound may eventually be appropriate, but a urinalysis would be recommended first. An MRI of the lumbar spine is not needed for this evaluation. It has long been established that there is an association between dermatomyositis, polymyositis, and malignancy. Although the malignancy risk is slightly higher in patients with dermatomyositis than with polymyositis, the malignancy association with both diseases is well established. The overall risk of cancer is highest in the first 3 years after the diagnosis of the myopathy, but it also continues over the individual's lifetime. Cancers most highly associated with inflammatory myopathy include lung, pancreatic, GI tract, non-Hodgkin lymphoma, and ovarian.

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QUESTION: 184

A 50-year-old man presents to your office with fatigue and weakness. He first noticed it a few weeks ago while trying to hang pictures with his wife. His legs have begun to ache as he walks up stairs. He has lost about 20 lbs in the last 3 months. Most recently, he has found that he is more constipated and has trouble rising from the commode. Your physical examination reveals modest proximal weakness, no articular swelling, rash, or any other pertinent findings. Blood work from a recent insurance examination revealed:
Sodium 142 meq/L; potassium 3.8 meq/L; chloride
107 meq/L; bicarbonate 29 meq/L; BUN
30 mg/dL; Cr 1.6 mg/dL; WBC 6.8; Hgb 13.6 g/dL;
HCT 40%; MCV 88.0 m3; platelets 240,000/mm3;
AST 200 U/L; ALT 250 U/L; alkaline phosphatase 70 U/L; bilirubin 0.3 mg/dL; ESR 40 mm/h

His CPK is 2400 and an EMG shows fibrillation potentials, positive sleep waves, and myotonic discharges. In addition to addressing his myositis, diagnostic testing should be performed to evaluate for the possibility of which of these?

Hodgkin lymphoma
testicular cancer
multiple myeloma
lung cancer
prostate cancer

Answer(s): D

Explanation:

The clinical features presented by the patient suggest a myopathy. It is often forgotten that serum transaminases are found in the muscle as well as the liver. Thus, a significant inflammatory myopathy may present with elevated serum transaminases in addition to symptoms. In this patient with normal alkaline phosphatase and bilirubin, initial measurement of the GGT would help rule out liver pathology and would be more appropriate initially than ultrasonography. Rhabdomyolysis may lead to renal dysfunction or even renal failure and a kidney ultrasound may eventually be appropriate, but a urinalysis would be recommended first. An MRI of the lumbar spine is not needed for this evaluation. It has long been established that there is an association between dermatomyositis, polymyositis, and malignancy. Although the malignancy risk is slightly higher in patients with dermatomyositis than with polymyositis, the malignancy association with both diseases is well established. The overall risk of cancer is highest in the first 3 years after the diagnosis of the myopathy, but it also continues over the individual's lifetime. Cancers most highly associated with inflammatory myopathy include lung, pancreatic, GI tract, non-Hodgkin lymphoma, and ovarian

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