DOI: http://dx.doi.org/10.18203/2320-6012.ijrms20203693

Non-purulent pharyngitis: adult still’s disease

Nagabhushanarao B. V., Narmada Vatti, Naveen Kumar Dannana, Surendra Konathala

Abstract


 Adult Still’s disease is rare and may present as pyrexia of unknown origin. Due to lack of expertise, diagnosis may be delayed inadvertently. The patient usually presents with spiked fever, polyarthralgia or arthritis, evanescent skin rash, non-purulent pharyngitis, lymphadenopathy and hepatosplenomegaly. Leukocytosis, predominantly of neutrophils, elevated erythrocyte sedimentation rate and C-reactive protein without obvious infection are the hallmarks of the disease. Delay in diagnosis may expose the patient to the side effects of antibiotics as they are repeatedly prescribed in view of elevated leukocytes. The majority of patients report pain in the throat without evidence of infection. This was an important clue to our diagnosis of this patient. Grossly elevated serum ferritin is diagnostic of adult onset still's disease. As the white cell counts are grossly elevated, a bone marrow examination to rule out hematological malignancy may be mandatory. Serum ferritin value has prognostic value too. Minor illness may respond to non-steroidal anti-inflammatory drugs (NSAIDs), but steroids are the mainstay of the treatment.  Methotrexate is of additional value for those presenting predominantly with arthritis. Anakinra, Infliximab and Tocilizumab are other options. Those patients presenting with severe disease and organ involvement require high dose intravenous steroids followed by high dose oral steroids.


Keywords


Adult Still’s disease, Non-purulent pharyngitis, Polyarthralgia, Serum ferritin, Spiky fever

Full Text:

PDF

References


Still GF. On a form of chronic joint disease in children. Med Chir Trans. 1897;80:47‐60.

Magadur-Joly G, Billaud E, Barrier JH, Pennec YL, Masson C, Renou P, et al. Epidemiology of adult Still's disease: estimate of the incidence by a retrospective study in west France. Ann Rheum Dis. 1995;54(7):587.

Gerfaud-Valentin M, Maucort-Boulch D, Hot A, Iwaz J, Ninet J, Durieu I, et al. Adult-onset still disease: manifestations, treatment, outcome, and prognostic factors in 57 patients. Med. 2014;93(2):91.

Calabro JJ, Marchesano JM. Fever associated with juvenile rheumatoid arthritis. N Engl J Med. 1967;276(1):11.

Gerfaud-Valentin M, Jamilloux Y, Iwaz J, Sève P. Adult-onset still's disease. Autoimmun Rev. 2014;13(7):708-22.

Bae CB, Jung JY, Kim HA, Suh CH. Reactive hemophagocytic syndrome in adult-onset still disease: clinical features, predictive factors, and prognosis in 21 patients. Med. 2015;94(4):e451.

Gerfaud-Valentin M, Maucort-Boulch D, Hot A, Iwaz J, Ninet J, Durieu I, et al. Adult-onset still disease: manifestations, treatment, outcome, and prognostic factors in 57 patients. Med. 2014;93(2):91.

Mert A, Ozaras R, Tabak F, Bilir M, Ozturk R, Ozdogan H, et al. Fever of unknown origin: a review of 20 patients with adult-onset Still's disease. Clin Rheumatol. 2003;22(2):89.

Elkon KB, Hughes GR, Bywaters EG, Ryan PF, Inman RD, Bowley NB et al. Adult-onset Still's disease. Twenty-year followup and further studies of patients with active disease. Arthritis Rheum. 1982;25(6):647.

Pouchot J, Sampalis JS, Beaudet F, Carette S, Décary F, Salusinsky-Sternbach M, et al. Adult Still's disease: manifestations, disease course, and outcome in 62 patients. Med. 1991;70(2):118.

Nguyen KH, Weisman MH. Severe sore throat as a presenting symptom of adult onset Still's disease: a case series and review of the literature. J Rheumatol. 1997;24(3):592.

Valente RM, Banks PM, Conn DL. Characterization of lymph node histology in adult onset still's disease. J Rheumatol. 1989;16:349.

Min JK, Cho CS, Kim HY, Oh EJ. Bone marrow findings in patients with adult Still's disease. Scand J Rheumatol. 2003; 32:119.

Ohta A, Yamaguchi M, Tsunematsu T. Adult Still's disease: a multicenter survey of Japanese patients. J Rheumatol. 1990;17:1058.

Schwarz-Eywill M, Heilig B, Bauer H, Breitbart A, Pezzutto A. Evaluation of serum ferritin as a marker for adult still's disease activity. Ann Rheum Dis. 1992;51(5):683.

Yamaguchi M, Ohta A, Tsunematsu T, Kasukawa R, Mizushima Y, Kashiwagi H, et al. Preliminary criteria for classification of adult Still's disease. J Rheumatol. 1992;19(3):424.

Fautrel B. Adult-onset still disease. Best Pract Res Clin Rheumatol. 2008; 22:773.