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E Hachulla

Publications and source records attributed to E Hachulla.

At least 199 records · Page 11Linked to original sources
PubMed1994

[Localized or systemic amyloidosis? Value and limitations of Iodine-123 labelled amyloid P component scintigraphy, role of biopsy of minor salivary glands].

We evaluated the value of serum amyloid P component scintigraphy (123I-SAP) and labial salivary gland biopsy in four patients with apparently localized amyloidosis. One patient had pharyngeal amyloidosis, the second laryngeal amyloidosis, the third retro-ocular amyloidosis and the fourth had carpal tunnel syndrome. Three patients (no 2, no 3, no 4) have abnormal whole body retention of 123I-SAP and positive labial salivary gland biopsy for amyloidosis. Only the first patient had effectively localized amyloidosis and local treatment was sufficient. Melphalan was given to the patients no 2 and no 3, colchicine was given to the patient no 4. Labial salivary gland biopsy and 123I-SAP scintigraphy may give positive argument for the diagnosis of systemic amyloidosis in patients with apparently localized amyloidosis.

Adult↗
PubMed1994

[Silicosis and primary Gougerot-Sjögren syndrome].

We report three cases of primary Sjögren's syndrome in silicotic coal miners. All patients fulfilled the diagnostic criteria for Sjögren's syndrome recently established by the European Community study group. One patient had cryoglobulinemia and polynevritis. Another had Raynaud's phenomenon, arthralgia, purpura and polynevritis. Capillary microscopy was normal in all the three patients. Antinuclear antibodies were detected only in one patient, who had also anti-SSa and anti-SSb antibodies. The prevalence of systemic sclerosis, rheumatoid arthritis and probably systemic lupus erythematosus is significantly higher after longstanding occupational exposure to silica. On the contrary, any case of Sjögren's syndrome was till now described in the course of pulmonary silicosis. The physiopathological mechanisms of these associations are misunderstood.

Aged↗
PubMed1994

[Vascular manifestations of dermatomyositis and polymyositis. Clinical, capillaroscopic and histological aspects].

Polymyositis is characterized by a T-cell-mediated and MHC-I-restricted cytotoxic process, whereas dermatomyositis is a primitively vascular disease with microangiopathy mediated by the complement C5b-9 membranolytic attack complex. We have tried to estimate the frequency of vascular abnormalities in polymyositis as defined by Bohan and Peter. We have retrospectively studied 17 patients with dermatomyositis and 15 patients with polymyositis. Vascular abnormalities have been defined by clinical, capillaroscopic and histologic (muscle biopsy and minor salivary glands biopsy) features. After clinical features, 5/17 dermatomyositis had a Raynaud's phenomenon, against 6/15 polymyositis. Digital necrosis has been observed for 2/17 dermatomyositis and 2/15 polymyositis. In capillaroscopy, 14/17 dermatomyositis had a microangiopathy with or no enlarged capillary loops, against 7/15 polymyositis. None of these polymyositis had enlarged capillary loops. The muscle biopsy showed a predominantly perivascular or perimysial inflammatory infiltrate (vascular process) for 10/16 dermatomyositis against 4/13 polymyositis; a perifascicular atrophy for 3/16 dermatomyositis against 2/13 polymyositis. The histological study of minor salivary glands, showed vascular lesions for 2/11 dermatomyositis and for 1/8 polymyositis. Finally, Bohan and Peter's classification is now inadequate to distinguish between dermatomyositis and polymyositis. Indeed, some dermatomyositis sine dermatitis, may exist and be recognized by their vascular features. To distinguish between dermatomyositis and polymyositis is important, to evaluate the risk of cancer which is more frequent in dermatomyositis.

Adult↗
PubMed1994

[Inappropriate ADH secretion syndrome and hypereosinophilia in Horton disease with stenosis of the subclavian arteries].

The authors describe a case of giant cell arteritis with subclavian involvement in association with a syndrome of inappropriate anti-diuretic hormone secretion and hypereosinophilia. Subclavian involvement, which may be the first symptom, occurs in five to twenty percent of giant cell arteritis. Hypereosinophilia, a frequent manifestation of specific granulomatosis vasculitis, is rarely described in association with giant cell arteritis. Dysfunction of the hypothalamo-pituitary axis such as inappropriate anti-diuretic hormone secretion or diabetes insipidus has been recently reported in giant cell arteritis. The syndrome of inappropriate anti-diuretic hormone secretion may either reveal the disease or occur during the evolution, in particular with gradual reduction of steroid treatment. It may be isolated, without clinical feature of arteritis and without an elevated erythrocyte sedimentation rate. This inappropriate anti-diuretic hormone secretion, probably due to ischemic involvement of the pituitary or hypothalamic region, always improves quickly after institution of steroid therapy.

Constriction, Pathologic↗
PubMed1994

Labial salivary gland biopsy assessment in rheumatoid vasculitis.

OBJECTIVES: To assess the vascular involvement in labial salivary gland (LSG) from patients with rheumatoid vasculitis (RV). METHODS: Forty seven patients with rheumatoid arthritis (RA) took part in a prospective study. Among them, 12 had proven RV. LSG biopsy was performed after local anaesthesia. RESULTS: Histological appearance of inflammatory vascular damage was observed in all but one patient with proven RV (92%). Inflammatory vascular involvement was also identified in LSG biopsy of seven patients with RA (20%) and only one patient in the control group (8%). A second specimen of LSG was studied after a mean treatment period of six months and failed to show any feature of inflammatory vascular involvement in three of the five cases that were analysed. CONCLUSIONS: The study emphasises the high incidence of immunopathological features of microvascular damage in patients with RV. LSG biopsy is minimally invasive and may be a potential useful tool for the diagnosis of RV especially when skin lesions are absent or impossible to biopsy. The assessment of the predictive value of positive LSG biopsy in RA requires a long term prospective study.

Aged↗
PubMed1994

Increased capillary permeability in systemic rheumatoid vasculitis: detection by dynamic fluorescence nailfold videomicroscopy.

OBJECTIVE: Several histological studies suggest a vascular involvement in rheumatoid arthritis (RA). We evaluated morphological and functional changes of skin capillaries in patients with RA. METHODS: We studied capillary permeability by nailfold fluorescence videomicroscopy in patients with RA with and without systemic vasculitis to establish the sensitivity, specificity, and the prognostic value of the technique for the diagnosis of systemic rheumatoid vasculitis (SRV). Nine patients with SRV, 22 patients with active RA without signs of vasculitis, and 16 controls were evaluated. RESULTS: No difference in conventional capillaroscopy was observed when we compared patients with SRV and RA. Using fluorescence videomicroscopy after intravenous injection of fluorescein sodium, we observed an increase of transcapillary dye diffusion in 9/9 patients with SRV, in 13/22 patients with RA without vasculitis and in 4/16 control patients. Our results give a sensitivity of 100% in SRV, a specificity of 44%, a positive predictive value of 34% and a negative predictive value of 100%. CONCLUSION: Our present study points out the functional alteration of the capillary wall in patients with RA with or without vasculitis and suggests the usefulness of dynamic fluorescence nailfold capillaroscopy in patients with RA when systemic vasculitis is suspected, not as a diagnostic tool, but because of the high negative predictive value of the technique.

Aged↗
PubMed1993

[Value of the assay of protein SAA and urinary antitrypsin activity in osteoarticular infections].

Erythrocyte sedimentation rate is normal in 20% to 25% of patients with discitis due to common pathogens. We evaluated serum amyloid A (SAA) protein and urinary trypsin inhibitory activity in osteoarticular infections comparatively with erythrocyte sedimentation rate and serum C-reactive protein in 20 patients including 14 with discitis due to common pathogens and 6 with septic arthritis. Assays were performed on D0, D8, D15, D30, and D60 after initiation of antimicrobial therapy. On D0, all four markers were significantly higher in patients with septic arthritis than in patients with discitis. C-reactive protein levels exhibited the fastest kinetics with a return to normal values within 15 days in both conditions. Urinary trypsin inhibitory activity was only slightly elevated in patients with discitis and returned to normal within 30 days in both conditions. Serum amyloid A levels required 30 to 60 days to return to normal. Erythrocyte sedimentation rate exhibited the slowest kinetics, with normal values being achieved only after 60 days. Although simple, rapid, and inexpensive, urinary trypsin inhibitory activity determination exhibits poor sensitivity. Serum amyloid A assay is not routinely available but may be a valuable parameter for monitoring patients whose erythrocyte sedimentation rate and C-reactive protein level are normal (as in 2 of our patients with discitis).

Arthritis, Infectious↗
PubMed1993

Labial salivary gland biopsy is a reliable test for the diagnosis of primary and secondary amyloidosis. A prospective clinical and immunohistologic study in 59 patients.

OBJECTIVE: Reports of the detection of amyloidosis by labial salivary gland (LSG) biopsy have been mostly anecdotal. The aim of this study was to assess the value of this method in the diagnosis of amyloidosis. METHODS: LSG biopsy tissues were studied with a combination method using Congo red stain and immunohistologic characterization using an antibody directed against the serum amyloid P (SAP) component. Electron microscopy was performed in all cases. In a prospective study, we evaluated 30 patients with biopsy-proven AA or AL amyloidosis. We compared these patients with a control group of 29 age-matched patients without clinical or biologic evidence of amyloid disease (14 had rheumatoid arthritis and 15 had plasma cell dyscrasia). RESULTS: In 26 of the 30 patients with known systemic amyloidosis, amyloid deposits were identified on LSG biopsy (sensitivity of 86%). In 1 of the remaining patients, amyloid deposits were identified on LSG biopsy and systemic amyloidosis was confirmed by abdominal fat biopsy and 123I-labeled SAP scintigraphy. CONCLUSION: This study emphasizes the high sensitivity of LSG biopsy in the diagnosis of amyloidosis, even in the absence of oral symptoms.

Adult↗
PubMed1993

Iodine-123-labelled serum amyloid P component scintigraphy in amyloidosis.

This study describes the results of scintigraphy with iodine-123-labelled serum amyloid P component (SAP) as a means of establishing the distribution of organ involvement in amyloidosis. The significance of 123I-SAP scans obtained in 15 patients with biopsy-proven AA or AL amyloidosis is discussed. Biopsy-proven amyloidosis was typically confirmed by scintigraphy, though such confirmation was not obtained in the kidneys in six patients with histological proof of extensive renal amyloid deposition. This lack of uptake may have been due to the accumulation of a major part of the 123I-SAP in the spleen and/or liver. Twenty-four hour whole-body retention of 123I-SAP was higher in patients with amyloidosis than in controls. Twenty-four hour tracer accumulation of the radioactivity in the extravascular compartment was notably greater in patients than in controls and appeared to be a good diagnostic criterion. We conclude that 123I-SAP scintigraphy may be helpful for the evaluation of organ involvement not only in patients with biopsy-proven amyloidosis but also when a biopsy cannot be performed or when a strong suspicion of amyloidosis exists in spite of repeated negative biopsies.

Amyloidosis↗
PubMed1993

[Diagnosis of amyloidosis in the elderly: value of the aspiration of the abdominal subcutaneous fat. Prospective study apropos of 100 cases].

Study of the presence of amyloid deposits on subcutaneous fat aspirates was carried out in 100 patients hospitalized in a geriatric unit. The test was positive in 15%, and in 4% of them, amyloid deposits were also seen on accessory salivary glands, confirmed by immunohistochemical staining. These results indicate that sub-cutaneous fat aspirate may be a useful test for an early diagnosis of amyloidosis.

Adipose Tissue↗
PubMed1993

[Rheumatoid polyarthritis in the elderly--rhizomelic pseudopolyarthritis: what differences?].

The authors have conducted a comparative retrospective study between polymyalgia rheumatic (N = 26) and rheumatoid arthritis in the elderly (N = 44), including HLA DRB1 genotype determination by PCR-RFLP analysis. No clinical nor biological differences were significant between the 2 groups of patients. However 70% of RA patients had one ore more susceptibility alleles (shared epitope hypothesis) and 50% in polymyalgia rheumatica.

Aged↗
PubMed1993

[Is the expression of TNF-alpha and cell adhesion molecules a marker of development of rheumatoid vasculitis?].

TNF-alpha, ICAM1, VCAM1, ELAM, CD44 and DR expression were studied (indirect immunoperoxydase methods) on labial salivary gland sections in 5 patients with proven rheumatoid vasculitis (before and after treatment), 5 patients with RA and 5 normal controls. TNF-alpha and cell adhesion molecules were particularly expressed in vasculitic areas of patients with systemic vasculitis and only before the onset of the treatment.

Biomarkers↗