- Email: [email protected]
Older Still…
Case Report
Older Still... Phillip Ferdinand, Timothy Warrener, Lauren Mitchell, Rasheed Zahir Lancet 2012; 379: 2312 Department of Acute Medicine, Wolverhampton New Cross Hospital, Wolverhampton, UK (P Ferdinand MRCP, T Warrener MBChB, L Mitchell MBChB, R Zahir FRCP) Correspondence to: Dr Phillip Ferdinand, Department of Acute Medicine, Wolverhampton New Cross Hospital, Wolverhampton, West Midlands, WV10 0QP, UK [email protected]
In April, 2011, a 52-year-old man presented to us with a 7-day history of fleeting joint pain and stiffness of his hips, right elbow, left shoulder, and lumbar region. The symptoms were worst in the evening and accompanied by myalgia in his quadriceps and gastrocnemius muscles. He had been unwell for 3 weeks with a swinging fever (>39°C), sore throat, cervical lymphadenopathy, and an intermittent pink rash across his chest, arms, and thighs. He had had no previous similar episodes and no recent diarrhoea, genitourinary symptoms, or red eyes. He denied any new sexual partners or travel to the tropics. On examination, he was febrile (temperature 39·2°C). He had swelling and reduced movement of his right elbow and left shoulder and discomfort on mobilisation of his lumbar spine. A striking pink rash covered his chest, arms, and thighs. There was no palpable lymphadenopathy or abdominal mass. Initial investigations showed a white cell count of 28×10⁹/L (neutrophils 26·9×10⁹/L), C-reactive protein 224 mg/L, ESR 91 mm/h, and normal renal and hepatic function. There was a delayed rise in alkaline phosphatase (360 IU/L) and alanine aminotransferase (48 IU/L). His serum ferritin was very high at 17 000 pmol/L. Chest radiograph was unremarkable. Serial blood and urine cultures grew no organisms, and broad-spectrum antibiotics were given. We detected no anti-nuclear antibodies, rheumatoid factor, antibody to cyclic citrullinated peptide, or extractable nuclear antigens. Epstein-Barr virus, cytomegalovirus, and parvovirus serology and screening for infectious mononucleosis and malaria were also negative. Thoracic, abdominal, and pelvic CT showed no fluid collections, abscesses, or evidence of malignancy. Transthoracic echocardiography did not reveal any valvular vegetations, and MRI of his Panel: Yamaguchi criteria for diagnosis of AOSD2 Major criteria • Fever ≥39°C for 1 week or longer • Arthralgia for 2 weeks or longer • Typical rash • Leucocytosis (>10×10⁹/L) with >80% granulocytes Minor criteria • Lymphadenopathy and/or splenomegaly • Liver dysfunction (raised serum aminotransferases) • Sore throat • Negative rheumatoid factor and antinuclear antibodies
spine showed no discitis. After exclusion of competing diagnoses, and completion of the diagnostic Yamaguchi criteria (panel), a diagnosis of adult-onset Still’s disease (AOSD) was made. Our patient was unresponsive to high-dose NSAIDs, but after treatment with prednisolone 60 mg daily for 8 weeks, his arthritic symptoms improved and the biochemical markers had normalised. He tolerated a graded reduction in prednisolone to 20 mg daily, with normal ESR and concentrations of CRP and ferritin. At last follow-up in November, 2011, he was well and had begun a phased return to work. With an incidence of 0·16 per 100 000,1 AOSD is a multi-system inflammatory disorder usually presenting in the second or third decade of life. It is diagnosed by use of the sensitive (96·2%) and specific (92·1%) Yamaguchi criteria.2 Typical features are a high swinging fever, polyarthritis, and an evanescent salmon-pink rash that is more prominent during episodes of fever. Although not part of the diagnostic criteria, ferritin concentrations are very high in many patients; together with its glycosylated form, ferritin might be important in future diagnostic criteria.3 The disease course is evenly split between monocyclic, polycyclic with remitting periods, and chronic forms.4 In mild disease, high-dose NSAIDs can be useful, but corticosteroids, combined with or substituted by methotrexate, are required in most cases. Other therapies including anakinra and tocilzumab have also been successful.5 Arthralgia, fever, and rashes are common symptoms; they have broad differential diagnoses, most of which are self-limiting or secondary to easily identifiable, common causes. Our case shows that persistence of these symptoms requires thorough investigation to facilitate diagnosis of disorders such as AOSD, which has a defined treatment that reverses otherwise disabling symptoms. Contributors PF, TW, LM, and RZ all looked after the patient and wrote the report. Written consent to publication was obtained. References 1 Magadur-Joly G, Billaud E, Barrier J, et al. Epidemiology of adult Still’s disease: estimate of the incidence by a retrospective study in west France. Ann Rheum Dis 1995; 54: 587–90. 2 Yamaguchi M, Ohta A, Tsunematsu T, et al. Preliminary criteria for classification of adult Still’s disease. J Rheumatol 1992; 19: 424–30. 3 Fautrel B, Le M, Saint-Marcoux B, et al. Diagnostic value of ferritin and glycosylated ferritin in adult onset Still’s disease. J Rheumatol 2001; 28: 322–29. 4 Catagay Y, Gul A, Catagay A, et al. Adult-onset Still’s disease. Int J Clin Pract 2009; 63: 1050–55. 5 Fautrel B. Adult-onset Still’s disease. Best Prac Res Clin Rheumatol 2008; 22: 773–92.
Exclusions: infection, malignancy, and rheumatic diseases. Classification requires at least five criteria, of which two must be major.
2312
www.thelancet.com Vol 379 June 16, 2012
Older Still... Phillip Ferdinand, Timothy Warrener, Lauren Mitchell, Rasheed Zahir Lancet 2012; 379: 2312 Department of Acute Medicine, Wolverhampton New Cross Hospital, Wolverhampton, UK (P Ferdinand MRCP, T Warrener MBChB, L Mitchell MBChB, R Zahir FRCP) Correspondence to: Dr Phillip Ferdinand, Department of Acute Medicine, Wolverhampton New Cross Hospital, Wolverhampton, West Midlands, WV10 0QP, UK [email protected]
In April, 2011, a 52-year-old man presented to us with a 7-day history of fleeting joint pain and stiffness of his hips, right elbow, left shoulder, and lumbar region. The symptoms were worst in the evening and accompanied by myalgia in his quadriceps and gastrocnemius muscles. He had been unwell for 3 weeks with a swinging fever (>39°C), sore throat, cervical lymphadenopathy, and an intermittent pink rash across his chest, arms, and thighs. He had had no previous similar episodes and no recent diarrhoea, genitourinary symptoms, or red eyes. He denied any new sexual partners or travel to the tropics. On examination, he was febrile (temperature 39·2°C). He had swelling and reduced movement of his right elbow and left shoulder and discomfort on mobilisation of his lumbar spine. A striking pink rash covered his chest, arms, and thighs. There was no palpable lymphadenopathy or abdominal mass. Initial investigations showed a white cell count of 28×10⁹/L (neutrophils 26·9×10⁹/L), C-reactive protein 224 mg/L, ESR 91 mm/h, and normal renal and hepatic function. There was a delayed rise in alkaline phosphatase (360 IU/L) and alanine aminotransferase (48 IU/L). His serum ferritin was very high at 17 000 pmol/L. Chest radiograph was unremarkable. Serial blood and urine cultures grew no organisms, and broad-spectrum antibiotics were given. We detected no anti-nuclear antibodies, rheumatoid factor, antibody to cyclic citrullinated peptide, or extractable nuclear antigens. Epstein-Barr virus, cytomegalovirus, and parvovirus serology and screening for infectious mononucleosis and malaria were also negative. Thoracic, abdominal, and pelvic CT showed no fluid collections, abscesses, or evidence of malignancy. Transthoracic echocardiography did not reveal any valvular vegetations, and MRI of his Panel: Yamaguchi criteria for diagnosis of AOSD2 Major criteria • Fever ≥39°C for 1 week or longer • Arthralgia for 2 weeks or longer • Typical rash • Leucocytosis (>10×10⁹/L) with >80% granulocytes Minor criteria • Lymphadenopathy and/or splenomegaly • Liver dysfunction (raised serum aminotransferases) • Sore throat • Negative rheumatoid factor and antinuclear antibodies
spine showed no discitis. After exclusion of competing diagnoses, and completion of the diagnostic Yamaguchi criteria (panel), a diagnosis of adult-onset Still’s disease (AOSD) was made. Our patient was unresponsive to high-dose NSAIDs, but after treatment with prednisolone 60 mg daily for 8 weeks, his arthritic symptoms improved and the biochemical markers had normalised. He tolerated a graded reduction in prednisolone to 20 mg daily, with normal ESR and concentrations of CRP and ferritin. At last follow-up in November, 2011, he was well and had begun a phased return to work. With an incidence of 0·16 per 100 000,1 AOSD is a multi-system inflammatory disorder usually presenting in the second or third decade of life. It is diagnosed by use of the sensitive (96·2%) and specific (92·1%) Yamaguchi criteria.2 Typical features are a high swinging fever, polyarthritis, and an evanescent salmon-pink rash that is more prominent during episodes of fever. Although not part of the diagnostic criteria, ferritin concentrations are very high in many patients; together with its glycosylated form, ferritin might be important in future diagnostic criteria.3 The disease course is evenly split between monocyclic, polycyclic with remitting periods, and chronic forms.4 In mild disease, high-dose NSAIDs can be useful, but corticosteroids, combined with or substituted by methotrexate, are required in most cases. Other therapies including anakinra and tocilzumab have also been successful.5 Arthralgia, fever, and rashes are common symptoms; they have broad differential diagnoses, most of which are self-limiting or secondary to easily identifiable, common causes. Our case shows that persistence of these symptoms requires thorough investigation to facilitate diagnosis of disorders such as AOSD, which has a defined treatment that reverses otherwise disabling symptoms. Contributors PF, TW, LM, and RZ all looked after the patient and wrote the report. Written consent to publication was obtained. References 1 Magadur-Joly G, Billaud E, Barrier J, et al. Epidemiology of adult Still’s disease: estimate of the incidence by a retrospective study in west France. Ann Rheum Dis 1995; 54: 587–90. 2 Yamaguchi M, Ohta A, Tsunematsu T, et al. Preliminary criteria for classification of adult Still’s disease. J Rheumatol 1992; 19: 424–30. 3 Fautrel B, Le M, Saint-Marcoux B, et al. Diagnostic value of ferritin and glycosylated ferritin in adult onset Still’s disease. J Rheumatol 2001; 28: 322–29. 4 Catagay Y, Gul A, Catagay A, et al. Adult-onset Still’s disease. Int J Clin Pract 2009; 63: 1050–55. 5 Fautrel B. Adult-onset Still’s disease. Best Prac Res Clin Rheumatol 2008; 22: 773–92.
Exclusions: infection, malignancy, and rheumatic diseases. Classification requires at least five criteria, of which two must be major.
2312
www.thelancet.com Vol 379 June 16, 2012