Recurrent fever of unknown origin (FUO): aseptic meningitis, hepatosplenomegaly, pericarditis and a double quotidian fever due to juvenile rheumatoid arthritis (JRA).
Abstract
BACKGROUND
Fever of unknown origin (FUO) has been defined as a fever of ≥101°F that persists for 3 weeks or more. It is not readily diagnosed
after 1 week of intensive in-hospital testing or after intensive outpatient or inpatient testing. Fevers of unknown origin
may be caused by infectious diseases, malignancies, collagen vascular diseases, or a variety of miscellaneous disorders. The
relative distribution of causes of FUOs is partly age-related. In the elderly, the preponderance of FUOs is attributable to
neoplastic and infectious etiologies, whereas in children, collagen vascular diseases, neoplasms, and viral infectious disease
predominate. The diagnostic approach to FUOs depends on a careful analysis of the history, physical findings, and laboratory
tests. Most patients with FUOs exhibit localizing findings that should direct the diagnostic workup and limit diagnostic possibilities.
The most perplexing causes of FUOs involve those without specific diagnostic tests, e.g., juvenile rheumatoid arthritis (JRA)
or adult Still's disease. In a young adult with FUO, if all of the cardinal symptoms are present, JRA may present either a
straightforward or an elusive diagnosis, if key findings are absent or if the diagnosis goes unsuspected.
METHODS
We present a 19-year-old man with a recurrent FUO. His illness began 3 years before admission and has recurred twice since.
In the past, he did not manifest arthralgias, arthritis, or a truncal rash. On admission, he presented with an FUO with hepatosplenomegaly,
aseptic meningitis, and pericarditis. An extensive diagnostic workup ruled out lymphoma and leukemia. Moreover, a further
extensive workup eliminated infectious causes of FUO appropriate to his clinical presentation, ie, tuberculosis, histoplasmosis,
brucellosis, Q fever, typhoid fever, Epstein-Barr virus, infectious mononucleosis, cytomegalovirus, human herpes virus (HHV)-6,
babesiosis, ehrlichiosis, viral hepatitis, and Whipple's disease.
RESULTS
The diagnosis of JRA was based on the exclusion of infectious and neoplastic disorders in a young adult with hepatosplenomegaly,
aseptic meningitis, pericarditis, and a double quotidian fever. With JRA, tests for rheumatic diseases are negative, as they
were in this case. The only laboratory abnormalities in this patient included elevated serum transaminases, a mildly elevated
erythrocyte sedimentation rate, and a moderately elevated level of serum ferritin.
CONCLUSION
Diagnostic fever curves are most helpful in cases where the diagnosis is most elusive, as was the case here. Relatively few
disorders are associated with a double quotidian fever, ie, visceral leishmaniasis, mixed malarial infections, right-sided
gonococcal acute bacterial endocarditis, and JRA. Because the patient received antipyretics during the first week of admission,
fever was not present. After infectious disease consultation during week 2 of hospitalization, antipyretics were discontinued,
and a double quotidian fever was present, which provided the key diagnostic clue in this case.
Links
Authors
Institution
Infectious Disease Division, Winthrop-University Hospital, Mineola, New York 11501, USA.
Source
Heart & lung : the journal of critical care 41:2 pg 177-80MeSH
Arthritis, Juvenile RheumatoidBody Temperature
Diagnosis, Differential
Fever of Unknown Origin
Hepatomegaly
Humans
Magnetic Resonance Imaging
Male
Meningitis, Aseptic
Pericarditis
Splenomegaly
Young Adult
Pub Type(s)
Case ReportsJournal Article
Language
eng
PubMed ID
21453973
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