French practical guidelines for the diagnosis and management of AA amyloidosis.

AA amyloidosis is secondary to the deposit of excess insoluble Serum Amyloid A (SAA) protein fibrils. AA amyloidosis complicates chronic inflammatory diseases, especially chronic inflammatory rheumatisms such as rheumatoid arthritis and spondyloarthritis; chronic infections such as tuberculosis, bronchectasia, chronic inflammatory bowel diseases such as Crohn's disease; and auto-inflammatory diseases including familial Mediterranean fever. This work consists of the French guidelines for the diagnosis workup and treatment of AA amyloidosis. We estimate in France between 500 and 700 cases in the whole French population, affecting both men and women. The most frequent organ impaired is kidney which usually manifests by oedemas of the lower extremities, proteinuria, and/or renal failure. Patients are usually tired and can display digestive features anf thyroid goiter. The diagnosis of AA amyloidosis is based on detection of amyloid deposits on a biopsy using Congo Red staining with a characteristic green birefringence in polarized light. Immunohistochemical analysis with an antibody directed against Serum Amyloid A protein is essential to confirm the diagnosis of AA amyloidosis. Peripheral inflammatory biomarkers can be measured such as C Reactive protein and SAA. We propose an algorithm to guide the etiological diagnosis of AA amyloidosis. The treatement relies on the etiologic treatment of the undelying chronic inflammatory disease to decrease and/or normalize Serum Amyloid A protein concentration in order to stabilize amyloidosis. In case of renal failure, dialysis or even a kidney transplant can be porposed. Nowadays, there is currently no specific treatment for AA amyloidosis deposits which constitutes a therapeutic challenge for the future.

[Therapeutic intensification and autologous stem cell transplantation in autoimmune diseases]. / Intensification thérapeutique et autogreffe de cellules souches hématopoïétiques pour le traitement des maladies auto-immunes.

THE PATHOPHYSIOLOGY of most autoimmune diseases is often poorly understood. EXPERIMENTAL CONSIDERATIONS and clinical experience suggest that high doses immunoablation followed by stem cell transplantation is a therapeutic option to consider for certain severe autoimmune disorders. THE CONCEPT OF RESTORING NORMAL IMMUNE REACTIVITY must in part br true since current results of 466 transplants (445 autologous, 21 allogeneic) patients suffering from various autoimmune diseases show a beneficial outcome in approximately 2/3 of the patients. TO IMPROVE THE EFFICACY AND SAFETY OF SUCH AN AGGRESSIVE PROCEDURE in patients with potentially affected vital organs by the underlying autoimmune disease, it is especially important to follow international consensus guidelines and to centrally collect clinical data for in depth analysis in the EBMT International Stem Cell Project for Autoimmune Disease in Basel, Switzerland. PHASE III STUDIES ARE RUNNING FOR SYSTEMIC SCLEROSIS (Astis, Autologous Stem cell Transplantation International Rheumatoid Arthritis Trial) started in 2003. A STUDY PROJECT IS PLANNED FOR MULTIPLE SCLEROSIS (Astims, Autologous Stem cell Transplantation International Multiple Sclerosis).

Hyaluronidase activity in serum of patients with monoclonal gammapathy.

Hyaluronidase and hyaluronic acid, two substances thought to be strongly implicated in carcinogenesis, were assessed in the plasma of 35 patients with newly documented monoclonal gammapathy and in 25 control patients. A significant increase was found in plasma hyaluronidase activity in the patients with monoclonal gammapathy. A statistically significant positive correlation was found between hyaluronidase activity and monoclonal immunoglobulin levels in plasma. An increase in serum hyaluronidase activities may be a response to the deleterious effect of hyaluronic acid in cell migration and tumor progression. Further studies are needed to assess the value of hyaluronidase activity as a marker of tumor progression.

[Hyperferritinemia]. / Les hyperferritinémies.

Many causes provoke elevated serum ferritin levels including not only iron overload, but also many pathological situations where hyperferritinemia or modified isoferritin profiles have no direct relationship with body iron stores. There are five general categories of hyperferritinemia: hemochromatosis, inflammatory syndromes, cytolysis, hemophagocytosis, Still's disease, and other etiologies.

[Uterine localization of periarteritis nodosa disclosed by fever of long duration]. / Localisation utérine d'une périartérite noueuse révélée par une fièvre au long cours.

We report the case of an 81 year-old woman admitted because of declining general health, fever and elevated sedimentation rate. Identification of a pelvic mass on the computed tomographic scan led to perform hysterectomy with a working diagnosis of ovarian malignancy. Histological examination revealed typical lesions of polyarteritis nodosa of uterus and fallopian tube vessels. Diffuse process of the vasculitis was suggested by persistence of fever and elevated erythrocyte sedimentation rate after hysterectomy. Treatment consisting of high-dose corticosteroids and pulses of cyclophosphamide resulted in prompt clinical and biological improvement. We discuss the incidence, the clinical features and the localized or diffuse nature of genital involvement in polyarteritis nodosa and other vasculitis.

Cauda equina compression by epidural lipomatosis in obesity. Effectiveness of weight reduction.

Epidural lipomatosis (EL) is characterized by abnormal accumulation of unencapsulated fat in the epidural space, and usually occurs as a complication of longterm steroid therapy. This condition, which may result in devastating neurologic complications, has also been reported without exogenous steroid intake. We describe a case of nonsteroid induced symptomatic EL associated with obesity, and emphasize the possibility of effective medical management of this entity with weight reduction instead of decompressive laminectomy whenever neurologic symptoms are mild and stable.

[Osteoarticular tuberculosis. Diagnostic contribution of local sampling]. / Tuberculose ostéoarticulaire. Apport diagnostique du prélèvement local.

Bone and joint tuberculosis have recently gained a renewal of interest, especially with the spread of HIV infection which may increase its frequency. Bone and joint locations of tuberculosis are pauci-bacillary often requiring local sampling in order to confirm the diagnosis and to initiate early therapy. From 1983 to 1992 we have studied 19 patients with bone and joint tuberculosis. Seventeen local sampling were performed: 12 biopsies and five abscess punctures. Pathological examination of samples disclosed diagnosis of tuberculosis in eight cases out of 12. Among the remaining four patients, direct smear was positive once, and cultures grew Mycobacterium tuberculosis in two, yielding the diagnosis in 11 out of the 12 patients. Bacteriological and pathological examinations were non contributive in only one patient. Microbiological examination of pus disclosed two positive direct smear and three positive cultures. Treatment lasted 9 to 18 months. The outcome was favourable in all patients.