Faculty Bibliography

Efforts continue to identify and consistently utilize those clinical, laboratory, imaging, and other features of rheumatoid arthritis that best reflect the disease process and its impact on individual patients. We seek descriptions that are accurate, reproducible, simple, sensitive, and predictive. Such assessments will lead to development of prognoses for individual patients and to more rational patient management. The past year has witnessed reemphasis of health status indexes (instruments) and other simple approaches to clinical assessment of patients, eg, use of standardized grip strength, button test, walk time, and modified articular indexes. Computed tomography and magnetic resonance imaging provided clinically important and otherwise unappreciated (but expensive) information about joint integrity and inflammatory disease with sensitivity and resolution considerably beyond conventional techniques. Laboratory assessment of patients included consideration or reconsideration of the utility of measurements of C-reactive protein, rheumatoid factors, immune complexes, complement receptors and complement activation products, antiperinuclear factors, trace elements, interleukins and interleukin receptors, soluble cell surface receptors, lymphoid cell phenotypes, and synovial immunohistology; all are important in the pathogenesis of rheumatoid arthritis and all have contributed variably to predicting patient outcomes. None were shown to be more clinically informative than erythrocyte sedimentation rate or C-reactive protein. The variables that have been associated with unfavorable prognosis for rheumatoid arthritis are also discussed. We hope that continued study will lead to identification and adoption of simple assessments that will prove to be powerful predictors of good or poor patient outcomes and stratification of patient risk. This uniform measure of disease assessment will improve judgments of potential benefits of therapeutic interventions.

Felty's syndrome, consisting of rheumatoid arthritis, leukopenia, and splenomegaly, has been recognized as a distinct clinical entity for more than 60 years. Clinical and laboratory manifestations of the condition are reviewed. The major sources of morbidity and mortality remain recurrent local and systemic infections. Immunogenetic analysis shows a strong association with HLA-DR4, in addition to DQ beta 3b and C4B null allele. Potential mechanisms of neutropenia are contrasted, including impaired granulopoiesis and neutrophil-immune complex interactions. Lithium carbonate and splenectomy may have a role in the treatment of fulminant disease. Maintenance therapy should be directed at control of the underlying inflammatory arthropathy. A syndrome of proliferation of large granular lymphocytes and neutropenia, associated with rheumatoid arthritis in 23% to 39% of cases, has been described recently. Cases of "pseudo-Felty's" syndrome are often confused with traditional Felty's syndrome, which has twice the prevalence. The clinical and laboratory distinctions between these two conditions are elaborated.

Two recent cases of Takayasu's arteritis illustrate the wide spectrum of disease presentation. The first patient demonstrated rapid development of ischemic manifestations; the second patient presented with septal panniculitis. Worldwide distribution of Takayasu's arteritis has increased.

The case of a 54-year-old woman with Felty's syndrome whose course was complicated by mucocutaneous lesions clinically typical of pyoderma gangrenosum is described. Necrotizing sinusitis and saddle nose deformity were distinctive clinical features. Lymphocytic vasculitis and rheumatoid nodule formation observed within panniculus at the base of a cutaneous lesion and in a nasal mucosal lesion were unexpected histopathologic findings.

We describe 2 cases of acute encephalopathy in patients with connective tissue disease caused by small doses of ibuprofen. In addition to aseptic meningitis, both patients had altered mental status and focal neurologic signs, ophthalmoplegia in one and hemiparesis in the other. The spectrum of neurologic manifestations of ibuprofen hypersensitivity is reviewed

A 29-year-old woman had an isolated, pupil-sparing third cranial nerve palsy. Serologic and CSF abnormalities and the subsequent course were consistent with systemic lupus erythematosus. Corticosteroid therapy resulted in improvement of ocular palsy within 4 weeks. The pathogenesis of cranial neuropathy in systemic lupus erythematosus and the unique presentation in this patient are discussed.

A woman 68 years of age had fever, malaise, diffuse lymphadenopathy, splenomegaly followed by abdominal pain, and diarrhea. A lymph node biopsy specimen showed nonspecific follicular hyperplasia. Symptoms were responsive initially to prednisone. Recurrent symptoms warranted colonic biopsy, which was consistent with Crohn's disease, and were responsive partially to prednisone and azulfidine. Because of progressive deterioration, a repeat lymph node biopsy was performed and showed the characteristic histologic feature of angioimmunoblastic lymphadenopathy (AILD). The evolution of the histopathologic features of the case is discussed, and gastrointestinal (GI) manifestations of AILD are reviewed. Although the GI tract is an unusual site for extra nodal AILD, colonic involvement can imitate the clinical and histologic features of inflammatory bowel disease.