Infections in a Woman With Adult-Onset Still's Disease

[Guest guest]

Infections in a Woman With Adult-Onset Still's Disease

from Medscape Rheumatology

Question

A 36-year-old female has had adult-onset Still's disease (AOSD) for 10

years. The disease has been in remission for 4 years without therapy. In the

meantime, pancytopenia and hepatitis C virus positivity developed. Liver

biopsy disclosed mild hepatitis, and bone marrow is scanty. Her clinical

problems are recurrent bacterial and viral infections. Your recommendation

for therapy, please.

Dusan Stefanovic, MD, PhD

Response

from J. Cush, MD, 02/19/2002

Treatment of AOSD is focused either on the systemic or articular disease.

Systemic disease (fever, rash, weight loss, hepatosplenomegaly, diffuse

lymphadenopathy, serositis, leukocytosis, high erythrocyte sedimentation

rate/C-reactive protein/ferritin) would initially depend on corticosteroids

to get rapid control of a sick patient. Nonsteroidal anti-inflammatory drugs

(NSAIDs) may be effective in a few patients, but most with a firm diagnosis

of AOSD will require prednisone 40-80 mg initially. Steroid-sparing therapy

should be begun soon in most patients and may include either methotrexate,

hydroxychloroquine, azathioprine, or tumor necrosis factor (TNF) inhibitors,

depending on disease severity and drug safety.

Treatment of the more indolent and chronically progressive articular disease

is similar to that for rheumatoid arthritis and may include methotrexate,

hydroxychloroquine, sulfasalazine, gold salts, azathioprine, etanercept,

infliximab, steroids, and NSAIDs as needed. But this patient does not have

either systemic or articular disease that would merit these therapies.

Patients with AOSD are not prone to recurrent infections (aside from risks

associated with steroids and other drugs) and are not at increased risk for

hepatitis C.

The case described appears to be largely a consequence of either chronic

hepatitis C infection or an as yet to be diagnosed additional malady. No

symptoms of active AOSD or arthritis are described. The author appears to be

looking for some therapy that might help the patient's recurrent infections

and possibly the hepatitis C infection, or in the least not affect the

course of her prior disease.

The patient should be evaluated for immunodeficiency that may lead to

recurrent bacterial and viral infections. She is not on drug therapy that

might be implicated or have disease activity (from hepatitis C or AOSD) that

may add to her infectious risk. An immunodeficiency work-up may include

tests for deficient complement components, immunoglobulins, human

immunodeficiency virus (HIV) type 1 or 2, etc. A search might be made for

coexistent active autoimmune disease (eg, systemic lupus erythematosus),

protein-losing enteropathy, or malignancy. Treatment would be predicated on

a more accurate diagnosis. For instance, if the patient had a demonstrable

immunoglobulin deficiency, then periodic treatment with intravenous gamma

globulin should prove efficacious. There is no literature to suggest that

treatment of such a patient with a TNF inhibitor, corticosteroids,

hydroxychloroquine, etc, would decrease the incidence or types of

infection.[1,2]

----------------------------------------------------------------------------

----

References

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

2.. Rosen FS, MD, Wedgwood JP. The primary immunodeficiencies. N

Engl J Med. 1995;333:431-440.

Visit the International Still's Disease Foundation Website

http://www.stillsdisease.org