Self Assessment Questions in Rheumatology
Yousaf Ali
Self Assessment Questions
in Rheumatology
Yousaf Ali MD, FACR
Assistant Clinical Professor of Medicine
Brown University
Rhode Island
Yousafali1@[Link]
ISBN: 978-1-934115-52-7 e-ISBN: 978-1-59745-497-1
DOI: 10.1007/978-1-59745-497-1
Library of Congress Control Number: 2008942270
© Humana Press a part of Springer Science+Business Media, LLC 2009
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Preface
This book is for postgraduate fellows, internists, and students interested in rheuma-
tology. It contains real cases and is designed to stimulate thought and further
reading in this rapidly evolving specialty.
I have included a series of common and uncommon cases that I have seen over
the past decade in a busy consultative practice and have inserted up-to-date refer-
ences and questions you may be asked on ward rounds or in clinic. My hope is that
it will be a useful adjunct for physicians preparing for examinations or entering the
field and will stimulate further interest.
I would like to acknowledge my mentors Pierre Bouloux, MD, Tom Cooney,
MD and thank Atul Deodhar, MD for his careful review of the manuscript.
This book is dedicated to my parents and wife Batool who have made many
sacrifices for which I remain eternally grateful.
Brooklyn, NY Yousaf Ali
v
Contents
Preface . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . v
Self Assessment Questions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1
Index . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 141
vii
Question 1
A 56-year-old female presents with arthralgias and fatigue. Lab work is unremark-
able apart from normocytic anemia and Howell Jolly bodies on peripheral smear. Her
examination reveals a blistering rash on the elbows.
What is the most likely diagnosis?
What are the other hematologic complications of this disease?
Yousaf Ali, Self Assessment Questions in Rheumatology, DOI: 10.1007/ 978-1-59745-497-1,
Humana Press, a part of Springer Science + Business Media, LLC 2009
1
Answer: Celiac disease (CD)
This disease is more common in Northern European ancestry characterized by
sensitivity to gluten. Patients present with symptoms of malabsorption, arthralgia,
skin rash, and hematologic disorders. Dermatitis herpeteformis is a blistering skin
rash seen in this condition. Howell Jolly bodies reflect nuclear remnants that persist
due to hyposplenism. Hematologic complications of CD include anemia due to
malabsorption of iron, B12, or folate; lymphoma, leucopenia, thromboembolism,
and IgA deficiency.
Halfdanarson TR, Litzow MR, Murray JA. Hematologic manifestations of celiac disease. Blood.
2007 Jan 15;109(2):412–21.
2
Question 2
A 35-year-old female is referred for evaluation of + antinuclear antibodies (ANA).
She is asymptomatic. Her lab work reveals ANA 1:320 homogeneous pattern;
extractable nuclear antigens are negative. Hematologic and renal function values are
normal and urinalysis is without sediment. Her examination is unremarkable apart
from a smooth nontender goiter. The past medical history is significant for
Hashimoto’s thyroiditis. No rash, synovitis, or serositis is observed.
What is the most likely diagnosis?
What further treatment or investigations are warranted?
Yousaf Ali, Self Assessment Questions in Rheumatology, DOI: 10.1007/ 978-1-59745-497-1,
Humana Press, a part of Springer Science + Business Media, LLC 2009
3
Answer: Hashimotos thyroiditis
The ANA is most likely of no clinical significance. Up to 46% of patients with
autoimmune thyroid disease have positive antinuclear antibodies. Assuming that
the patient remains asymptomatic no further intervention is warranted.
Petri M, Karlson EW, Cooper DS, Ladenson PW. Autoantibody tests in autoimmune thyroid
disease: a case-control study. J Rheumatol. 1991 Oct;18(10):1529–31.
4
Question 3
You are asked to evaluate a 74-year-old male with joint pain. He was recently
admitted after a bout of diverticulitis. On examination he has polyarticular syno-
vitis, and arthrocentesis reveals multiple intracellular uric acid (UA) crystals diagn-
ostic of gout. Lab values: UA = 5.5 mg/dl, WBC = 10.5, HB = 11.5, Plts = 550, and
creatinine = 2.8 mg/dl.
How would you best manage this patient’s gout? Why is the uric acid normal?
Yousaf Ali, Self Assessment Questions in Rheumatology, DOI: 10.1007/ 978-1-59745-497-1,
Humana Press, a part of Springer Science + Business Media, LLC 2009
5
Answer: Oral corticosteroids
This is a common inpatient scenario. A stable patient with renal failure is admitted
and undergoes a stressful procedure that triggers gout. Treatment of acute gout
involves high-dose NSAID, colchicine, or corticosteroids. In a patient with renal
failure and diverticular inflammation, NSAIDs and colchicine should be avoided
since they are poorly tolerated. A monoarticular presentation could be injected with
local intraarticular corticosteroid assuming that the cultures are negative. In this
patient with polyarticular gout a short burst of oral corticosteroid is the best option.
Uric acid levels fall and are often normal during an attack and should not be used
as a diagnostic test.
Terkeltaub RA. Clinical practice. Gout. N Engl J Med. 2003 Oct 23;349(17):1647–55.
6
Question 4
A 55-year-old female with longstanding Sjogrens syndrome (SS) presents with new
onset of lethargy, hypokalemia, and nephrocalcinosis. Her metabolic profile reveals
an anion gap metabolic acidosis, hypokalemia, and alkaline urine. A skeletal survey
reveals diffuse osteopenia.
What complication has occurred?
Yousaf Ali, Self Assessment Questions in Rheumatology, DOI: 10.1007/ 978-1-59745-497-1,
Humana Press, a part of Springer Science + Business Media, LLC 2009
7
Answer: Distal type 1 renal tubular acidosis (RTA)
This is a rare but important complication of SS. Distal RTA occurs due to failure to
acidify urine to a pH < 5.3. This results in anion gap acidosis, hypokalemia, neph-
rocalcinosis, and bone demineralization. The lymphocytes that invade the tubular
epithelial cells are CD8-positive, i.e., cytotoxic T cells and similar to those found
in the salivary glands of patients with Sjögren’s syndrome. The same immunologi-
cal process is probably operative in the renal tubulointerstitial tissue as in the
salivary glands to induce the characteristic tissue changes of Sjögren’s syndrome.
Matsumura R, Kondo Y, Sugiyama T, Sueishi M, Koike T, Takabayashi K, Tomioka H, Yoshida S,
Tsuchida H. Immunohistochemical identification of infiltrating mononuclear cells in tubulointersti-
tial nephritis associated with Sjögren’s syndrome. Clin Nephrol. 1988 Dec;30(6):335–40.
Moutsopoulos HM, Cledes J, Skopouli FN, Elisaf M, Youinou P. Nephrocalcinosis in Sjögren’s
syndrome: a late sequela of renal tubular acidosis. J Intern Med. 1991 Aug;230(2):187–91.
Wrong OM. Immune-related potassium-losing interstitial nephritis: a comparison with distal renal
tubular acidosis. QJM 1993;86(8):513–42.
8
Question 5
A 47-year-old patient with Crohn’s disease presents for evaluation of new onset
arthritis. She has a 20-year history of colitis managed with sulfasalazine and local
corticosteroid suppositories. Infliximab at 5 mg/kg was recently commenced due to
persistent disease activity. She arrives with new onset polyarticular joint pain. On
examination she has synovitis of the small joints of the hands and a warm knee
effusion. Lab work: WBC = 2.8, Hb = 11.2 g/dl, platelets = 554, ESR = 66,
ANA = 1:640, RF = 54, and renal and liver studies normal.
What is the most likely diagnosis?
What further tests can confirm this?
How would you manage this patient?
Yousaf Ali, Self Assessment Questions in Rheumatology, DOI: 10.1007/ 978-1-59745-497-1,
Humana Press, a part of Springer Science + Business Media, LLC 2009
9
Answer: Infliximab-induced SLE
This patient presents with a new polyarticular flare after commencing infliximab.
The differential diagnosis is between Crohn’s related arthropathy and drug-induced
SLE. The latter is more likely given the leucopenia and +ANA. Infliximab has been
reported to induce antinuclear antibodies and can cause drug-induced SLE. Prompt
discontinuation of the drug is warranted. Further testing includes checking
antibodies to histone protein, extractable nuclear antigens, serum complements,
differential cell count to look for lymphopenia, and renal function. Typically drug-
induced SLE does not involve major organ systems.
Watts RA. Musculoskeletal and systemic reactions to biological therapeutic agents. Curr Opin
Rheumatol. 2000 Jan;12(1):49–52.
10