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We conducted a retrospective observational study to describe a cohort and identify the prognostic factors in adult-onset Still disease (AOSD). Patients enrolled in this retrospective chart review fulfilled either Yamaguchi or Fautrel criteria. Candidate variables were analyzed with logistic unadjusted and adjusted regression models.Fifty-seven patients were seen in the internal medicine (75%) and rheumatology (25%) departments over a mean period of 8.4 years. The median time to diagnosis was 4 months. The course of AOSD was monocyclic in 17 patients, polycyclic in 25, and chronic in 15. The assessment of glycosylated ferritin (GF) in 37 patients was correlated with early diagnosis. Nine 18F-fluorodeoxyglucose positron emission tomography (18FDG-PET) scans identified the lymph nodes and glands as the main sites of hypermetabolism. Complications were frequent (n = 19), including reactive hemophagocytic syndrome (n = 8). None of the 3 deaths could be attributed to AOSD. Corticosteroid dependence, as predicted by a low GF level, occurred in 23 patients (45%). A quarter of the patients received tumor necrosis factor-α blockers or anakinra with good tolerance. Fever >39.5°C was predictive of monocyclic AOSD, while arthritis and thrombocytopenia were associated with chronic and complicated AOSD, respectively. The youngest patients had the highest risks of resistance to first-line treatments.AOSD remains difficult to diagnose. Mortality is low despite frequent complications. GF and 18FDG-PET scans were of value in the diagnostic approach. The condition in highly symptomatic patients evolved to systemic AOSD, whereas more progressive patterns with arthritis predicted chronic AOSD.

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Transverse myelitis may reveal SLE or occur more than 10 years after SLE diagnosis. The initial severity of the neurological flare (with paraplegia) is the main prognostic marker. The study provides arguments for cyclophosphamide use.

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BACKGROUND. The authors investigated how lymphopenia and low serum albumin levels correlate with the prognosis of patients with carcinoma of unknown primary (CUP). METHODS. Univariate and multivariate prognostic factor analyses were conducted in a population of 317 consecutive patients with CUP who were evaluated at the Cross Cancer Institute of Edmonton, Alberta, Canada, from 1998 to 2004. RESULTS. The results from multivariate analysis showed that patients who had a performance status ≥2 (using the World Health Organization scale), a high overall comorbidity score (on the Adult Comorbidity Evaluation 27), liver metastasis, elevated serum lactate dehydrogenase (LDH) levels, lymphopenia (defined as an absolute lymphocyte count ≥0.7 × 109/L), and low serum albumin levels had a worse prognosis. Based on the observation that the presence of liver metastasis and low serum albumin levels were the most powerful adverse prognostic factors, a classification scheme was delineated that took those 2 variables into account. A group of good‐risk patients (no liver metastasis and normal serum albumin levels) and a group of poor‐risk patients (liver metastasis and/or low serum albumin levels) were identified with median survivals of 371 days and 103 days, respectively (P < .0001). This classification was validated further in an independent data set of 124 patients who were evaluated at 2 French cancer centers: Among those patients, the median survival was 378 days in the good‐risk group and 90 days in the poor‐risk group (P < .0001). The new prognostic model substantially outperformed the previous standard prognostic model, which was based on performance status and serum LDH levels. CONCLUSIONS. Lymphopenia and low serum albumin levels were identified as 2 new independent markers of prognosis in patients with CUP. Although the authors confirmed the validity of the previous prognostic model, they developed and validated a more powerful, simple model based on the 2 most powerful adverse prognostic factors: liver metastasis and low serum albumin levels. These findings were confirmed in an independent cohort of patients with CUP, and consideration of the authors' improved prognostic model for survival of patients with CUP is warranted. Cancer 2006. © 2006 American Cancer Society.

BACKGROUND.The authors conducted a comprehensive review of the efficacy of 2‐deoxy‐2‐[F‐18]fluoro‐D‐glucose positron emission tomography (FDG‐PET) in the detection of primary tumors in patients with disseminated carcinoma of unknown primary site.METHODS.Ten studies (involving a total of 221 patients) tat were published between 1998 and 2006 were reviewed. Each study evaluated the role of FDG‐PET in the detection of unknown primary tumors after a conventional diagnostic workup. Although 94% of patients had a single site of metastases, the studies otherwise were very heterogeneous in the studied population, study design, and additional diagnostic workup.RESULTS.In 41% of patients, FDG‐PET detected primary tumors that were not apparent after conventional workup. In this group of patients, the overall sensitivity, specificity, and accuracy rates of FDG‐PET in detecting unknown primary tumors were 91.9%, 81.9%, and 80.5%, respectively. FDG‐PET imaging also led to the detection of previously unrecognized metastases in 37% of patients. Lung cancers represented 59% of the detected tumors. FDG‐PET had a notably high false‐positive rate (58.3%) in tumors of the lower digestive tract. FDG‐PET altered the clinical management in 34.7% of patients. Most of those patients (53%) received specific chemotherapy for lung and pancreatic cancers; whereas 12% received specific therapy for breast, ovarian, and prostate cancers; and 14% underwent surgery with curative intent.CONCLUSIONS.FDG‐PET was an efficient method for detecting primary tumors that were undetected by other modalities and was sensitive for the detection of previously unrecognized metastases. FDG‐PET significantly changed clinical management in approximately one‐third of the patients studied. Cancer 2007. © 2006 American Cancer Society.

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