There are few reports of amyloidosis diagnosed by deliberate biopsy of accessory salivary glands. Usually, a biopsy performed for dry mouth syndrome reveals an unsuspected amyloidosis. We report the case of 2 patients with lambda-type light chain monoclonal gammapathy complicated by generalized amyloidosis and in whom biopsy of the accessory salivary glands showed signs of amyloidosis. In the first patient accessory salivary gland biopsy was performed because these glands were enlarged, and the monoclonal dysglobulinaemia was subsequently diagnosed by serum immunoelectrophoresis. In the second patient with nephrotic syndrome, renal biopsy could not be carried out owing to the presence of a renal malformation; amyloidosis was confirmed by periumbilical fat aspiration, and a systematic biopsy of accessory salivary glands also showed evidence of amyloidosis. Biopsy of accessory salivary glands seems to be a particularly simple and safe method to detect generalized amyloidosis in patients with chronic inflammatory disease or monoclonal dysglobulinaemia.

Aged↗

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[Double heart valve replacement disclosing antiphospholipid syndrome].

In patients with systemic lupus erythematosus (SLE) heart valve lesions are usually discovered at echocardiography; their haemodynamic repercussions are uncommon, and valve replacement is exceptional. We report the case of a woman who had undergone aortic and mitral valve replacement before antiphospholipid antibodies were found associated with 4 ARA criteria of SLE. Histopathological examination confirmed the diagnosis of Libman-Sachs specific endocarditis. The presence of antiphospholipid antibodies leads to a discussion of their role in the physiopathology of the heart valve lesions and vascular accidents that occurred in this patient. The overlap observed between the diagnostic criteria of SLE and those of primary antiphospholipid syndrome is discussed. Heart valve lesions may be one of the modes of access to the antiphospholipid syndrome.

Adult↗

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[Peripheral nerve diseases disclosing Horton's disease (2 cases)].

Peripheral nerves are rarely involved in Horton's disease, and when peripheral neuropathy predominates in the clinical complex it is sometimes a source of diagnostic errors. We report two cases of giant cell arteritis revealed by peripheral neuropathy.

Aged↗

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[Adult Still's disease. A too often unrecognized illness. A study of a series of 11 cases].

The authors report eleven new cases of adult Still's disease diagnosed during the last 5 years. This rare, though not exceptional, disease of unknown pathogenesis is difficult to diagnose in the absence of specific sign; it is in fact diagnosed by elimination. The clinical, laboratory and anatomical findings, as well as the treatment and outcome of these 11 cases are described, and this is followed by a discussion of the nosological, therapeutic and above all prognostic problems raised by the disease.

Adolescent↗

PubMedDate not supplied

[Autoimmune thrombopenic purpura revealing primary Sjögren's syndrome. 2 cases].

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Adult↗

PubMedDate not supplied

Prognostic factors and long-term evolution in a cohort of 133 patients with giant cell arteritis.

OBJECTIVE: Survival in patients with giant cell arteritis (GCA) has generally been found to be similar to that of the general population. The aim of our study was to assess outcome and survival of different subgroups of patients with GCA in relation to clinical, biological data or treatment modalities. METHODS: From 1977 and 1995, 176 patients were treated in the Department of Internal Medicine for GCA. The patient, family or local practitioner were contacted prior to the study (July-October 1995). Treatment modalities and follow-up were obtained for 133 patients. All patients (except 11) had 3 or more 1990 ACR classification criteria for GCA. The 11 patients with 2 criteria had a positive temporal biopsy and were included in the study. RESULTS: Relapse during corticosteroid tapering treatment was observed in 83 patients (62.4%) with a mean 1.57 relapses per patient. No correlation was found in age, sex, initial dose or type of steroid used (i.e. prednisone or prednisolone). Only a slight correlation in the initial erythrocyte sedimentation rate (ESR) was observed (p < 0.01, r = 0.23). In 56 patients free of treatment (mean treatment duration: 40 months), 27 (48%) developed a relapse of the disease 1 to 25 months later. No correlation was found in age, sex, initial dose of steroid, number of relapses during treatment, or initial ESR. Survival analysis was performed using the Kaplan-Meier and Mantel-Menszel methods for comparison of groups. At the time of the study, 41 patients had died (30.7%). A significant reduction of survival was found with the presence of permanent visual loss vs absence (p = 0.04), in patients who required more than 10 mg/d of glucocorticoid (p < 0.001) at 6 months treatment and in patients treated with prednisone (vs prednisolone) (p < 0.01). However, these factors were not independently associated with survival in the multivariate analysis. CONCLUSION: Relapse was observed in 62.4% of the patients during corticosteroid tapering (correlated with initial ESR). A relapse of the disease was also observed in 48% of patients 1 to 25 months after the end of the treatment and was associated with prednisolone use. Long term survival was better in patients with no initial ocular manifestations, in patients who took less than 10 mg/day of corticosteroids at 6 months of the treatment and in patients treated with prednisolone.

Aged↗

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[Study of static transcutaneous oximetry before amputation in patients with arteritis].

Transcutaneous oximetry has been used to define the level of amputation in arteritic patients, with discrepant results. We have studied preoperative statix oximetry in 33 arteritic patients at the Leriche-Fontaine Stage IV, who underwent 36 amputations (thighs = 6, Legs+Symes = 14, transmetatarsal = 7, toes = 9). Oximetry included the measurement, at the level of amputation, of the transcutaneous partial oxygen pressure (tc pO2), of the tissue oxygenation ratio (TOR), preferably with a precordial electrode, of the tc pO2 gain after oxygen inhalation and of the gain ratio with the reference electrode. Two patients died postoperatively, the amputation stump did not heal in another 8 patients. The tc pO2 value was 36.6 +/- 16.2 mm Hg in the healed group and 21.1 +/- 19.9 mm Hg in the non healed group. The TOR respectively was 71.2 +/- 32.7% and 38.6 +/- 29.9% in these two groups. These differences were statistically significant. The difference between the two groups in the measurements made after the oxygenation test was not statistically significant. With a tc pO2 threshold at 26 mm Hg, the sensitivity was 73% and the specificity 75%, the positive predictive value 90%, the negative predictive value 46% and the value of the test 73.5%. The thresholds calculated to be 56% for TOR, 11 mm Hg for the gain and 32% for the gain ratio, did not improve the performances of oximetry. In our study, the tc pO2 was the parameter that best allowed predicting healing was obtained with smaller values than the threshold. Other elements such as the general condition and diabetes have played a role in the prognosis.

Aged↗

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Serum amyloid A concentrations in giant-cell arteritis and polymyalgia rheumatica: a useful test in the management of the disease.

A prospective clinical study of 23 patients with giant-cell arteritis (GCA) and/or polymyalgia rheumatica (PMR) was undertaken in order to assess the behaviour of the non-specific markers of the disease activity, the erythrocyte sedimentation rate (ESR) and other acute phase markers, particularly the C-reactive protein (CPR) and serum amyloid A apolipoprotein (apo SAA) levels during induction of disease remission by prednisone therapy, and possible further recurrence of GCA and/or PMR. The apo SAA measurement is more sensitive than the CRP measurement in determining disease activity (97% and 61%, respectively). The specificity of apo SAA is greater than ESR in the determination of inactive disease (86% and 77%, respectively). In some cases with clinically active disease the ESR and CRP were normal, whereas the apo SAA was always elevated. We conclude that the apo SAA measurement in combination with clinical data and other laboratory parameters may be useful in the management of GCA and/or PMR.

Acute-Phase Proteins↗

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[Necrotizing angiitis].

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Female↗

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[T-lymphocyte diseases. Physiopathological and nosologic concepts (excluding cancer and graft rejection)].

The T-lymphocyte is at the heart of the physiopathological mechanism of most dysimmunity affections. Furthermore, some cases of granulomatosis, the immediate consequence of macrophagic disorder, come within the framework of T-lymphocyte diseases, épicritical centre of the complex process of cellular immunity. By reference to new knowledges about various actions of T-lymphocytes and their interactions with lymphocytes B and macrophages, and by reference to classic pathologic findings of diseases of immunity, the author's propose a new classification of these diseases, suggesting therapeutic reasearch in consideration of effect of drugs, especially immunostimulant treatments, on the immune system.

Antibody Formation↗

PubMedDate not supplied