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 Table of Contents  
CASE REPORT
Year : 2015  |  Volume : 2  |  Issue : 2  |  Page : 93-95

Adult-Onset Still's Disease: An Unusual Cause of Fever


Department of Internal Medicine, University Teaching Hospital, University of Zambia School of Medicine, Lusaka, Zambia

Date of Submission02-Jan-2015
Date of Acceptance12-Mar-2015
Date of Web Publication20-May-2015

Correspondence Address:
Dr. Owen Ngalamika
Department of Internal Medicine, University Teaching Hospital, University of Zambia School of Medicine, Lusaka
Zambia
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DOI: 10.4103/2384-5147.157433

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  Abstract 

Adult-onset Still's disease (AOSD) is a rare auto-inflammatory syndrome. In Zambia, like most parts of Africa, fever is usually caused by infections (e.g., tuberculosis) and infestations (e.g., malaria). AOSD represents one of the few febrile conditions that are not due to microbial infections or parasitic infestations. It therefore cannot be managed with antibiotics or antihelminthics. Generally, patients are <35-year-old, febrile, have arthralgias, and an evanescent maculopapular rash. Treatment of AOSD requires the use of immunosuppressant drugs. This is the first report of a patient with AOSD in Zambia. It is one of the very few cases to be reported in Africa.

Keywords: Adult-onset Still′s disease, auto-inflammatory syndrome, fever, microbial infections, parasitic infestations


How to cite this article:
Fwoloshi S, Trollip S, Ngalamika O. Adult-Onset Still's Disease: An Unusual Cause of Fever. Sub-Saharan Afr J Med 2015;2:93-5

How to cite this URL:
Fwoloshi S, Trollip S, Ngalamika O. Adult-Onset Still's Disease: An Unusual Cause of Fever. Sub-Saharan Afr J Med [serial online] 2015 [cited 2021 Jan 25];2:93-5. Available from: https://www.ssajm.org/text.asp?2015/2/2/93/157433


  Introduction Top


Adult-onset Still's disease (AOSD) is a rarely encountered systemic inflammatory condition of unknown etiology. It's characterized by spiking fever, an evanescent salmon-colored maculopapular rash, and arthritis of large and small joints. [1] The color description of the rash may be difficult to notice in dark-skinned populations. Other associated features include sorethroat, fatigue, night sweats, lymphadenopathy, serositis, hepatosplenomegaly, raised acute phase proteins, and neutrophillic leukocytosis. Pathogenesis of AOSD is presumed to be cytokine-driven, predominantly interleukin-1 (IL-1), which is increased in sera and target tissues. IL-1 promotes bone and cartilage destruction, pyrexia, exanthems, and anorexia. IL-6 is also overproduced and promotes the synthesis of acute phase proteins (e.g., ferritin). There is also increased expression of IL-18 in the liver, which promotes inflammation leading to elevated transaminases. Prognosis is usually favorable, and most patients respond well to immunosuppressant drugs. However, it's very important to recognize AOSD early to prevent complications and improve patient's quality of life. In this report, we present a case of AOSD that was initially treated as severe malaria and sepsis.


  Case Report Top


A 30-year-old female was referred from a Primary Health Care Centre to the University Teaching Hospital (UTH) for suspected severe malaria. She presented with a 2 week history of joint pains, daily fevers-predominantly nocturnal, and a rash. She also gave a history of poor appetite, a sore throat, fatigue, headache, and night sweats. She was referred because her symptoms were worsening despite receiving antimalarials and intravenous antibiotics for 1-week. Her family history was unremarkable. At about the age of 9-11 years, she had recurrent joint pains and presumed to have had sickle cell disease until laboratory tests proved otherwise.

On admission, she was febrile (39.2°C), had an urticarial rash (on thighs, arms, and face), with tender fingers, knees, and elbows. No joint deformity was noted. Her Pulse was 88 bpm and blood pressure was 111/77 mmHg. The rest of the examination was unremarkable. An impression of febrile illness was made, Ceftriaxone and paracetamol were commenced. She was then investigated for malaria, pulmonary tuberculosis, sepsis, and rheumatoid arthritis. The table below shows the laboratory results of the initially ordered investigations [Table 1].
Table 1: Laboratory results of investigations ordered upon admission of the patient

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The laboratory results, a poor response to antibiotics, and the patient's symptoms and signs led to the suspicion of AOSD as the most plausible diagnosis for this patient. Serum ferritin was immediately ordered (1759 ng/ml - very high), antibiotics were stopped, and a moderate Prednisolone dose (40 mg/day) was commenced. Low-dose Methotrexate (5 mg/week) was also commenced as a steroid-sparing agent. The patient's symptoms waned and she was discharged 3 days later. The patient returned for a follow-up visit 2 weeks later and was asymptomatic. The methotrexate dose was then raised to 10 mg/week and a tapering of Prednisolone was begun. Six weeks later, the patient was still asymptomatic, and her serum ferritin dropped to 50 ng/ml. She continues to be followed up at UTH.


  Discussion Top


Adult-onset Still's disease is one of the principle disorders considered under auto-inflammatory syndromes. There is only a handful of documented cases in Africa. Though there can be new onset in adults, it mostly occurs in children. [2] Our patient gave a history of having an undiagnosed joint disorder in childhood. Our assumption is that the childhood condition was most probably juvenile idiopathic arthritis.

During her admission, the patient had a fluctuating course of feeling better in the morning while her symptoms worsened at night. An infectious etiology was ruled out after administering antimalarials and trying several antibiotics with insignificant effect. A notable observation was the concurrent onset of symptoms. Even though the joint pains were almost always present at any time of the day, they worsened at night and were associated with the onset of fever and the itchy rash.

Both anti-nuclear antibody test and rheumatoid factor were negative. A negative rheumatoid factor excluded rheumatoid arthritis as a cause of the polyarthritis. Autoinflammatory syndromes like AOSD lack significant autoantibodies. [3] Our patient also had a neutrophilic leukocytosis.

Other documented manifestations of AOSD are deep vein thrombosis, [4] neurological disorders, atypical cutaneous features, [5] and acute respiratory distress syndrome. Three patterns of disease are described; monocyclic (self-limited pattern with complete remission within 2-4 weeks), Polycyclic/intermittent (symptomatic flares separated by periods of remission ranging from 2 weeks to 2 years), and a chronic polyarthritis pattern. [6] The monocyclic and polycyclic phenotypes are highly symptomatic while the chronic subtype has an indolent course with paucity of systemic symptoms. [7]

Adult-onset Still's disease is a diagnosis of exclusion. Yamaguchi criteria are the most widely used in diagnosing AOSD. Our patient met all the four major plus three minor criteria. Ferritin is a marker of inflammation that is markedly raised but not specific for AOSD. Other conditions with hyperferritinemia include septic shock, antiphospholipid syndrome, and macrophage activation syndrome. [8] Serum ferritin levels are directly associated with AOSD disease activity. [9] In the acute phase, our patient had serum ferritin levels more than 10 times the upper limit of normal. As expected, the serum ferritin levels dropped drastically and fell within the normal range upon responding to treatment.

Our patient went into remission after a few weeks of corticosteroid and methotrexate, which are considered the first-line drugs in the treatment of moderate to severe AOSD. [10] Methotrexate is also useful in preventing destructive arthritis. [10] In patients who are refractory to corticosteroids and other steroid sparing immunosuppressive agents, other options of proven efficacy include anakinra (IL-1 receptor antagonist) and tocilizumab (IL-6 inhibitor).

This case report illustrates an unusual cause of fever in a region of the world where fever is common and mostly due to the high burden of infectious diseases. Therefore, it is not surprising that microbial infections were the conditions initially sought for in this patient. Nevertheless, she presented with the typical cluster of symptomatology and laboratory findings of AOSD. Despite AOSD being a rare condition, clinicians are advised to have a high index of suspicion in patients presenting with spiking fever, joint pains, and an evanescent rash.

 
  References Top

1.
Cozzi A, Papagrigoraki A, Biasi D, Colato C, Girolomoni G. Cutaneous manifestations of adult-onset Still′s disease: A case report and review of literature. Clin Rheumatol 2014. [Epub ahead of print].  Back to cited text no. 1
    
2.
Cush JJ. Autoinflammatory syndromes. Dermatol Clin 2013;31:471-80.  Back to cited text no. 2
    
3.
Efthimiou P, Flavell RA, Furlan A, Gasbarrini G, Gava A, Koné-Paut I, et al. Autoinflammatory syndromes and infections: Pathogenetic and clinical implications. Clin Exp Rheumatol 2008;26:S53-61.  Back to cited text no. 3
    
4.
Morita H, Nishiwaki H, Nagayama Y, Yoshimura A. Portal vein thrombosis in adult-onset Still′s disease: A case report and literature review. Rheumatol Int 2009;29:1515-8.  Back to cited text no. 4
    
5.
Affleck AG, Littlewood SM. Adult-onset Still′s disease with atypical cutaneous features. J Eur Acad Dermatol Venereol 2005;19:360-3.  Back to cited text no. 5
    
6.
Pouchot J, Vinceneux P. Diagnosis, progression and prognosis, aetiology and treatment of adult-onset Still′s disease. Presse Med 2004;33:1019-26.  Back to cited text no. 6
    
7.
Gerfaud-Valentin M, Jamilloux Y, Iwaz J, Sè]ve P. Adult-onset Still′s disease. Autoimmun Rev 2014;13:708-22.  Back to cited text no. 7
    
8.
Rosário C, Zandman-Goddard G, Meyron-Holtz EG, D′Cruz DP, Shoenfeld Y. The hyperferritinemic syndrome: Macrophage activation syndrome, Still′s disease, septic shock and catastrophic antiphospholipid syndrome. BMC Med 2013; 11:185.  Back to cited text no. 8
    
9.
Mehta B, Efthimiou P. Ferritin in adult-onset Still′s disease: Just a useful innocent bystander? Int J Inflam 2012;2012:298405.  Back to cited text no. 9
    
10.
Manger B, Rech J, Schett G. Use of methotrexate in adult-onset Still′s disease. Clin Exp Rheumatol 2010;28 Suppl 61:S168-71.  Back to cited text no. 10
    



 
 
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