Adult Still's disease: part I. Manifestations and complications in sixty-five cases in France.

Masson C  Le Loet X  Liote F  Renou P  
 Dubost JJ  Boissier MC  Brithmer L  
 Bregeon C  Audran M  

In: Rev Rhum Engl Ed (1995 Dec) 62(11):748-57

ISSN: 0035-2659

DESIGN: a retrospective multicenter study conducted in France
 identified 65 cases of adult Still's disease. Data were recorded on a
 standardized questionnaire validated by the Inflammatory Joint
 Disease and Immunorheumatology Committee of the Societe Francaise     de  Rhumatologie.

OBJECTIVES: (1) To compare clinical and laboratory
 findings in our patients with those reported in earlier studies,
 particularly two large series from Canada and Japan, respectively;
 (2) to describe the systemic and visceral complications associated
 with adult Still's disease.

RESULTS: as compared with the two above- mentioned series, our study group included more patients who had  experienced onset of their disease after the age of 35 years and  fewer patients with involvement of the liver, spleen, or lymph nodes.  Rates of occurrence of arthritis, myalgia, sore throat, pleuritis,  pericarditis, and abdominal pain were significantly higher in the  Canadian series than in the other two series. Arthritis was absent in
 one fourth of our patients. Life-threatening complications included
 "Still's hepatopathy", disseminated intravascular coagulation (with
 hemophagocytosis in some cases), and "Still's myocarditis".
 CONCLUSION: differences in the expression of adult Still's disease
 were found between patients from Canada, France, and Japan. Adult
 Still's disease can be responsible for life-threatening
 complications.


Adult Still's disease. Part II. Management, outcome, and prognostic
 factors.

In: Rev Rhum Engl Ed (1995 Dec) 62(11):758-65

ISSN: 0035-2659

DESIGN: a multicenter study conducted in France identified 65 cases
 of adult Still's disease. Follow-up exceeded one year in 52 cases.
 

OBJECTIVES: were as follows: 1) to describe treatments used; 2) to
 analyze disease course patterns; 3) to study joint alterations; 4) to
 determine whether any characteristics present within the first six
 months of onset were of prognostic significance.

RESULTS: aspirin was  ineffective. Indomethacin ensured satisfactory control in eight  patients. Corticosteroid therapy was required in 88% of cases. Among  patients followed up for more than one year, half developed  radiologic joint alterations; 23% had monocyclic systemic disease,  38.5% had polycyclic systemic disease and 38.5% had chronic articular  disease. More than half of the patients (58%) had more than one
 systemic flare. Polyarthritis at onset and involvement of the
 proximal limb joints were significantly predictive of chronic
 articular disease, whereas isolated arthralgia was predictive of
 monocyclic or polycyclic systemic disease. Oligoarthritis was not
 predictive of the outcome.

CONCLUSION: the knowledge that  polyarthritis or proximal limb joint involvement within six months of  onset is predictive of chronic joint disease may have important  therapeutic implications.

Institutional address:
       Rheumatology Department
       Angers
       France.