Board Simulation: Pediatric Rheumatology



Board Simulation: Pediatric Rheumatology


Steven Spalding



QUESTIONS



1. You are seeing a 3-year-old white male, who has recently moved to your city, for fever. Going through his chart, you notice that he has been seen about every 4 weeks for fevers for the last 6 months. During the history, his mother reports that he has fever every 26 to 27 days “like clockwork” and that these fevers are associated with sore throat, swollen glands, and abdominal pain. His fevers remain in the 102°F to 103°F range for 4 to 5 days and are minimally responsive to acetaminophen and ibuprofen. He is completely healthy between febrile episodes. On examination, his growth parameters are normal. He is febrile at 39.6°C and is illappearing. There is oropharyngeal erythema, tonsillar hypertrophy, posterior pharyngeal ulcers, anterior cervical lymphadenopathy, and abdominal tenderness to palpation. Of the following, which is the most likely laboratory finding in this patient?


a) Tumor necrosis factor receptor super family 1A gene mutation


b) Elevated erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP)


c) Elevated ferritin


d) Mevalonate kinase gene mutation


e) Mediterranean fever gene mutation

View Answer

Answer

The answer is b. Periodic fever, aphthous stomatitis, pharyngitis, and adenopathy, or PFAPA syndrome is a relatively common cause of recurrent fever in children, but its true prevalence is unknown. Although the exact cause has yet to be determined, this syndrome results in stereotypic attacks of fever lasting 4 to 5 days recurring on a fairly regular interval of approximately 4 to 5 weeks. Although not contained in the acronym, abdominal pain accompanies febrile episodes in up to 60% of patients. Children with PFAPA are completely healthy between attacks and display normal growth and development. During attacks, inflammatory markers such as the ESR and CRP are typically elevated, but return to normal between attacks. Children who remain ill between febrile episodes, have atypical growth and/or development, or persistently elevated inflammatory markers are more likely to have a more serious cause of their recurrent fevers. In general, the prognosis of children with PFAPA is good with nearly 40% either entering spontaneous remission or reduction in frequency and severity of attacks within 2 to 5 years of symptom onset.



2. Which therapeutic option has shown the greatest efficacy in treatment of the condition for the patient in question 1?


a) Adenotonsillectomy


b) Prednisone 1 mg/kg by mouth at onset of symptoms


c) Cimetidine 10 mg/kg by mouth four times a day


d) Infliximab 5 mg/kg every 4 weeks


e) Methotrexate 15 mg/m2 subcutaneously every week

View Answer

Answer

The answer is a. There are several treatment options for PFAPA. A single dose of oral prednisone or prednisolone (0.5-2 mg/kg) has been shown to abort attacks if given at the first sign of fever in >90% of PFAPA patients. However, almost 20% of patients who use prednisone to manage their attacks suffer an increase in the frequency and severity of attacks. Cimetidine and colchicine have also been used to prevent PFAPA episodes with success in 33% of patients. The medication must be given every day and missing a single dose can result in an attack. Adenotonsillectomy has been gaining popularity in the management of this condition. To date, close to 100 patients with PFAPA have undergone tonsillectomy reported in the literature, which has resulted in complete cessation of attacks in virtually 100% patients within 12 months after the procedure. There have been no
reported adverse events. Infliximab and methotrexate have not been used for treatment of PFAPA.



3. A 16-year-old white female presents for evaluation of fatigue. For the last 4 months, she has been experiencing intermittent muscle and joint pain, in addition to chronic daily headaches. The pain wakes her from sleep and she often feels tired upon waking. She has not been to school in 2 weeks and takes a 3-hour nap every day. Her examination is normal except for tenderness over her trapezii, second ribs, medial tibial tuberosities, and gluteus medius. What treatment is most likely to result in long-term improvement in this patient?


a) Pregabalin (Lyrica) 50 mg three times a day


b) Referral to pain medicine


c) Physical therapy with cognitive behavioral therapy


d) Oral prednisone at 1 mg/kg per day for 5 days followed by a 2-week taper


e) Oral naproxen at 10 mg/kg twice a day

View Answer

Answer

The answer is c. Juvenile fibromyalgia syndrome (jFMS) is an increasingly recognized condition. Patients with jFMS complain of a combination of the following six symptoms: fatigue, disordered and nonrestorative sleep, chronic headaches, nonspecific gastrointestinal complaints, widespread musculoskeletal pain above and below the waist, and chronic anxiety or tension. In addition to these somatic complaints, these patients have exquisite tenderness at characteristic musculoskeletal tender points on physical examination. Several medications have recently been marketed as treatment for adult fibromyalgia, including pregabalin and duloxetine. At the time of publication, there have been no prospective studies examining the safety or efficacy of these medications in the treatment of jFMS. Experts in jFMS recommend a multidisciplinary treatment plan including intensive physical therapy, cognitive behavioral therapy, and improved sleep hygiene. Adjunctive use of amitriptyline (<75 mg) in combination with a multidisciplinary care plan may also be beneficial. Longitudinal studies on patients with jFMS are few so prognosis cannot be stated with accuracy. However, successful participation in and completion of a multidisciplinary care plan usually results in sustained return of function but pain may persist for years.



4. A 15-year-old female presents to your office for evaluation of fatigue, low-grade fevers, headaches, abdominal pain after eating, and pain in her left arm with exercise. On examination she is obese and mildly hypertensive (132/89 mm Hg), but your nurse also notes a 15 mm Hg difference in systolic blood pressure between arms. The remainder of your exam is normal. What is the next most appropriate step in your evaluation?


a) Magnetic resonance angiography of aorta and great vessels


b) Referral to physical therapy


c) Referral to neurology


d) Initiate amitriptyline at 25 mg by mouth before bed


e) Initiate enalapril 10 mg once a day

View Answer

Answer

The answer is a. This patient’s signs and symptoms are suggestive of vasculitis, specifically Takayasu’s arteritis (TA). TA is a large-vessel vasculitis causing stenotic and rarely aneurismal changes in the aorta and its branches. The thoracic aorta and its branches are commonly involved but the abdominal aorta and renal arteries may be more frequently affected in children. Children with TA may present with constitutional symptoms such as fatigue and malaise or hypertension. In addition, children are less likely than adults to complain of carotidynia (pain over course of internal carotid arteries) and claudication. Recently proposed classification criteria for childhood TA require demonstration of angiographic abnormalities of the aorta or its primary branches using magnetic resonance, computed tomography, or conventional angiography plus at least one of the four following features:

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Jul 5, 2016 | Posted by in CRITICAL CARE | Comments Off on Board Simulation: Pediatric Rheumatology

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