Relapse of Wegener's granulomatosis. Concerning a case after 20 years of remission.

Immunosuppressive drugs have transformed the prognosis of systemic Wegener's granulomatosis. Nowadays, the main residual problem is illness relapses, for which management is largely undefined. We describe the case of a patient, aged 47 in 1977. The diagnosis of Wegener's granulomatosis was made when faced with polyarthralgias, cutaneous vasculitis, rhinitis, dyspnea, hemoptysis and global decline of her physical condition. The treatment associated high-dose corticotherapy and intramuscular cyclophosphamide for 1 year. This treatment led to a complete remission. Twenty years later, the patient was hospitalized for reoccurrence of rhinitis, dyspnea and right knee effusion associated with biological inflammatory syndrome, renal insufficiency and antibodies against polymorphonuclear neutrophil cytoplasm, type c-ANCA. Chest CT-scan disclosed parenchymal infiltrates. Wegener relapse was diagnosed and the combination of three methylprednisolone perfusions followed by oral prednisone (1 mg/kg/d) and a monthly bolus of cyclophosphamide led to a new remission. Nevertheless, 4 months after beginning the treatment the patient died from an infectious complication (Pneumocystis carinii and aspergillosis). Relapses of Wegener's granulomatosis are frequent and difficult to predict. Moreover, some cases occur very early. The remarkable efficiency of cyclophosphamide to induce remission is however shaded by the high rate of relapse. Other drugs are studied to identify more efficient therapy, able to both induce remission and prevent relapses, but reliable data are still missing to determine the best therapeutic regimen.

Diagnostic value of ferritin and glycosylated ferritin in adult onset Still's disease.

OBJECTIVE: To determine the usefulness of serum ferritin and glycosylated ferritin (GF) levels in diagnosing adult onset Still's disease (AOSD). METHODS: We performed a retrospective multicenter study of 205 patients who had ferritin and GF assays in one hospital laboratory. Records of all patients were reviewed, and a standardized questionnaire used to extract all data available at the time of the assay. The clinicians' final diagnosis was also recorded. Patients were classified as having "certain AOSD" (based on Yamaguchi's criteria) or a control disease. The concordance of ferritin and GF levels with final diagnosis was evaluated. RESULTS: In total 49 AOSD and 120 control patients were eligible. The mean ferritin value was significantly higher in the AOSD group (4,752 +/- 9,599 microg/l) than in the control group (1,571 +/- 3,807 microg/l), p = 0.029. GF was significantly lower in AOSD patients (15.9 +/- 11.9%) than in the control group (31.5 +/- 18.7%), p < 0.001. The combination of a GF level of < or = 20% with ferritin above the upper limit of normal yielded a sensitivity of 70.5% and specificity of 83.2%. The combination of a GF level < or = 20% with ferritin 5 times normal produced a sensitivity of 43.2% and specificity of 92.9%. This latter combination allowed an AOSD diagnosis to be ruled out for 6 of the 8 control patients who met Yamaguchi's positive criteria. CONCLUSION: Ferritin and GF levels are powerful diagnostic markers of AOSD. They may be helpful in clinical practice for excluding differential diagnoses.

Corticosteroid sparing effect of low dose methotrexate treatment in adult Still's disease.

OBJECTIVE: Adult Still's disease (ASD) is a rare chronic polyarthritis, usually treated with corticosteroid therapy. Because some patients become dependent on high dose prednisone or are refractory to that treatment, and because adverse events are frequent with corticosteroid, we evaluated the efficacy of low dose methotrexate (MTX) as a second-line drug. METHODS: We retrospectively studied 26 patients with ASD treated with low dose MTX because their disease was either resistant to or dependent on corticosteroids. RESULTS: The group included 13 women and 13 men, with a mean age of 32.6 years at onset of ASD. Mean disease duration at the beginning of MTX treatment was 59.9 mo (range 7 to 444). Evaluation took place at the maximum followup, which averaged 48.9 mo (range 8 to 136). The mean dose of MTX was 11.5+/-3.6 mg/week (range 7.5 to 17.5). Twenty-three patients responded to MTX; 18 had complete remission. No difference was seen between patients with or without extraarticular manifestations. Leukocyte and neutrophil counts and erythrocyte sedimentation rate were significantly reduced (p = 0.0001). Daily prednisone intake decreased by 69% (21.5 mg) (p = 0.0001). Eleven patients were able to stop taking corticosteroids. One patient with AA amyloidosis renal failure died of neutropenia: this was the only serious adverse event. CONCLUSION: MTX is an effective second-line treatment of ASD that does not respond to prednisone. It allows significant reduction of corticosteroid doses, which is beneficial to these patients, who have frequent and numerous corticosteroid related adverse events.

Magnetic resonance appearance of monoclonal gammopathies of unknown significance and multiple myeloma. The GRI Study Group.

STUDY DESIGN: A prospective multicenter study. OBJECTIVES: To evaluate the use of magnetic resonance imaging, in the differentiation between monoclonal gammopathies of unknown significance and multiple myeloma. SUMMARY OF BACKGROUND DATA: Although multiple myeloma has been studied extensively with magnetic resonance imaging, to the authors' knowledge, no study has evaluated the clinical interest of magnetic resonance imaging in the differentiation between monoclonal gammopathies of unknown significance and multiple myeloma. METHODS: The magnetic resonance examinations of the thoracolumbar spine in 24 patients with newly diagnosed monoclonal gammopathies of unknown significance were compared with those performed in 44 patients with newly diagnosed nontreated multiple myeloma. RESULTS: All findings on magnetic resonance examination performed in patients with monoclonal gammopathies of unknown significance were normal, whereas findings on 38 (86%) of the 44 magnetic resonance examinations performed in patients with multiple myeloma were abnormal. CONCLUSION: Magnetic resonance imaging can be considered as an additional diagnostic tool in differentiating between monoclonal gammopathies of unknown significance and multiple myeloma, which may be helpful when routine criteria are not sufficient. An abnormal finding on magnetic resonance examination in a patient with monoclonal gammopathies of unknown significance should suggest the diagnosis of multiple myeloma after other causes of marrow signal abnormalities are excluded. Magnetic resonance imaging also may be proposed in the long-term follow-up of monoclonal gammopathies of unknown significance when a new biologic or clinical event suggests the diagnosis of malignant monoclonal gammopathy.

[Normal phosphorus and calcium metabolism during pregnancy and breast feeding]. / Métabolisme phosphocalcique normal pendant la grossesse et l'allaitement.

Calcium homeostasis is stressed considerably during pregnancy and lactation. Important regulatory mechanisms are needed both for meeting the fetal requirement for calcium and for protecting the maternal skeleton from excessive resorption. For the past 10 years, more and more publications have deal with the involved mechanisms of regulation. The authors have reviewed these publications and tried to answer important questions: is there some irreversible bone loss related to pregnancy and/or lactation? How can such bone loss could be prevented?

[Pathological phosphorus and calcium metabolism during pregnancy and breast feeding]. / Métabolisme phosphocalcique pathologique pendant la grossesse et l'allaitement.

The occurrence of calcium and vitamin D deficiencies is enhanced during pregnancy and lactation. The presentation of parathyroid diseases is altered during gestation. A peculiar form of osteoporosis, pregnancy-associated osteoporosis, may be observed. Fetus and newborns suffer the consequences of all these disorders. The authors have reviewed the early detection and treatment of these diseases and emphasized their prevention.

Diversity of opinions on the management of gout in France. A survey of 750 rheumatologists.

Gout is a common disease. Although effective treatments are available for gout, there is some disagreement as to how they should be used. To study prescription patterns in gout, we conducted a questionnaire survey among 2520 rheumatologists. Seven hundred and fifty completed questionnaires were returned over a two-month period. Among respondents, 35.4% worked in a private office, 21% in a hospital and 43.6% in both. The most widely prescribed treatments in acute gout attack were colchicine alone (63%), colchicine with a nonsteroidal antiinflammatory drug (NSAID) (31.7%) and NSAID alone (5.2%), with significant variations according to the type of practice. Mean duration of treatment in acute gout was 18 +/- 16.8 days (range, 3-180 days). Mean time interval between the attack and initiation of therapy with a xanthine oxidase inhibitor was 21.6 +/- 17.2 days (range 0-180); here also, significant variations were seen according to the type of practice. Concomitant symptomatic therapy was prescribed in 97.3% of cases, for a mean duration of 54 +/- 55.4 days (range 2-365). Thirty per cent of responders never prescribed uricosuric agents. The estimated rate of occurrence of treatment-induced attacks increased with the reported interval between the attack and initiation of urate-lowering therapy. Our data demonstrate that French rheumatologists have widely diverging views on how to treat gout. Whether a waiting period is needed between an acute attack and initiation of urate-lowering therapy, and how long this period should be, are unsettled issues that deserve to be studied.