24‑year old male with febricity, multi‑organ dysfunction and fast progressing ARDS

Česká verzia

Authors: J. Chvojka; A. Kroužecký; J. Raděj; R. Sýkora; T. Karvunidis; I. Novák; M. Matějovič
Authors place of work: I. interní klinika Lékařské fakulty UK a FN Plzeň, přednosta doc. MU Dr. Martin Matějovič, Ph. D.
Published in the journal: Vnitř Lék 2009; 55(10): 991-994
Category: Case Reports

Summary

A 24‑year- old man presented with cough, sore throat, fever, maculopapulous exanthema, pericardial and pleural effusion. Despite extensive evaluation neither infectious, autoimmune, hematological nor oncological disorders were revealed. Broad spectrum antibiotic and subsequently corticosteroid treatment failed to resolve the symptoms. Multiorgan failure with rapid progress of acute respiratory distress syndrome and circulatory failure developed and patient died. Adult onset Still’s disease (AOSD), a diagnosis considered in this patient, is a rare disease with unknown prevalence, pathogenesis and etiology. Clinically it is characterized by spiking fever, arthritis, rash, and impairment of multiple organs. There is no single diagnostic test for AOSD. Rather, the diagnosis is based on the clinical criteria and requires the exclusion of infectious, neoplastic, and other autoimmune diseases. Rarely the course of the disease can be rapidly progressive to death. Treatment includes the use of non‑steroid antirheumatic drugs and corticosteroids. Limited data suggest that biological agents (e. g. anti‑TNF‑α, anti‑IL‑1), rituximab or intravenous immunoglobulins might be promising for the treatment of severe cases.

Key words:
fever – exanthema – multiorgan dysfunction – ferritin – adult onset Still’s disease

Zdroje

1. Bywaters EG. Still’s disease in the adult. Ann Rheum Dis 1971; 30: 121– 133.

2. Magadur- Joly G, Billaud E, Barrier JH 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– 590.

3. Wakai K, Ohta A, Tamakoshi A et al. Estimated prevalence and incidence of adult Still’s disease: findings by a nationwide epidemiological survey in Japan. J Epidemiol 1997; 7: 221– 225.

4. Uson J, Peña JM, del Arco A et al. Still’s disease in a 72‑year- old man. J Rheumatol 1993; 20: 1608– 1609.

5. Steffe LA, Cooke CL. Still’s disease in a 70 year-old woman. JAMA 1983; 249: 2062– 2063.

6. Efthimiou P, Paik PK, Bielory L. Diagnosis and management of adult onset Still’s disease. Ann Rheum Dis 2006; 65: 564– 572.

7. Colebunders R, Stevens WJ, Vanagt E et al. Adult Still’s disease caused by Yersinia enterocolitica infection. Arch Intern Med 1984; 144: 1880– 1882.

8. Pouchot J, Sampalis JS, Beaudet F et al. Adult onset Still’s disease: manifestations, disease course and outcome in 62 patients. Medicine (Baltimore) 1991; 70: 118– 136.

9. Sampalis JS, Medsger TA jr, Fries JF et al. Risk factors for adult onset Still’s disease. J Rheumatol 1996; 23: 2049– 2054.

10. Calabro JJ, Marchesano JM. Fever associated with juvenile rheumatoid arthritis. N Engl J Med 1967; 276: 11– 18.

11. Elkon KB, Hughes GR, Bywaters EG et al. Adult onset Still’s disease. Twenty‑year follow‑up and further studies of patients with active disease. Arthritis Rheum 1982; 25: 647– 654.

12. 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: 592– 597.

13. McFarlane M, Harth M, Wall WJ. Liver transplant in adult Still’s disease. J Rheumatol 1997; 24: 2038– 2041.

14. Pouchot J, Sampalis JS, Beaudet F et al. Adult Still’s disease: manifestations, disease course, and outcome in 62 patients. Medicine (Baltimore) 1991; 70: 118– 136.

15. Ohta A, Yamaguchi M, Tsunematsu Tet al. Adult Still’s disease: a multicenter survey of Japanese patients. J Rheumatol 1990; 70: 1058– 1063.

16. Cheema GS, Quismorio FP jr. Pulmonary involvement in adult- onset Still’s disease. Curr Opin Pulm Med 1999; 5: 305– 309.

17. Suleiman M, Wolfovitz E, Boulman N et al. Adult onset Still’s disease as a cause of ARDS and acute respiratory failure. Scand J Rheumatol 2002; 31: 181– 183.

18. Arlet JB, Le TH, Marinho A et al. Reactive haemophagocytic syndrome in adult- onset Still’s disease: a report of six patients and a review of the literature. Ann Rheum Dis 2006; 65: 1596– 1601.

19. Boki KA, Tsirantonaki MJ, Markakis K et al. Thrombotic thrombocytopenic purpura in adult Still’s disease. J Rheumatol 1996; 23: 385– 387.

20. Cush JJ. Adult- onset Still’s disease. Bull Rheum Dis 2000; 49: 1– 4.

21. Schwarz- Eywill M, Heilig B, Bauer H et al. Evaluation of serum ferritin as a marker for adult Still’s disease activity. Ann Rheum Dis 1992; 51: 683– 685.

22. Van Reeth C, Le Moel G, Lasne Y et al. Serum ferritin and isoferritins are tools for diagnosis of active Still’s disease. J Rheumatol 1994; 21: 890– 895.

23. Akritidis N, Giannakakis I, Giouglis T. Ferritin levels and response to treatment in patients with Adult Still’s disease (letter). J Rheumatol 1996; 23: 201– 202.

24. Coffernils M, Soupart A, Pradier O et al. Hyperferritinemia in adult onset Still’s disease and the hemophagocytic syndrome. J Rheumatol 1992; 19: 1425– 1427.

25. Křivanová A, Adam J, Mayer J et al. Teplota nejasné etiologie: příčiny a diagnostický postup. Vnitř Lék 2007; 53: 169– 178.