Does vascular endothelial growth factor in the rheumatoid synovium predict joint destruction? A clinical, radiological, and pathological study in 12 patients monitored for 10 years.

OBJECTIVE: Synovial angiogenesis is at the epicenter of rheumatoid pannus development and is largely dependent on vascular endothelial growth factor (VEGF). We sought to determine whether the VEGF level in rheumatoid synovial tissue is a marker for disease severity. PATIENTS AND METHODS: Twelve patients with rheumatoid arthritis (RA) underwent a clinical and radiological evaluation at the time of a synovial biopsy done during joint surgery required by RA progression (T1) and, on average, 10 years later (T2). Immunohistochemistry was used to detect and quantitate VEGF in the synovial biopsy taken at T1. RESULTS: VEGF labeling was seen on endothelial cells and macrophages in all 12 synovial biopsies. The amount of endothelial-cell VEGF labeling (assessed semi-quantitatively) was significantly correlated with Larsen score progression during the 10-year follow-up. The amounts of endothelial cell or macrophage VEGF labeling was not correlated with the joint count, radiological stage of the biopsied joint or progression of this stage, Larsen scores at T1 or T2, presence of rheumatoid factor, or presence of extra-articular manifestations. CONCLUSION: Our results suggest that the amount of VEGF in the rheumatoid synovium may be a marker for joint destruction in patients with RA.

In the rheumatoid pannus, anti-filaggrin autoantibodies are produced by local plasma cells and constitute a higher proportion of IgG than in synovial fluid and serum.

IgG anti-filaggrin autoantibodies (AFA) are the most specific serological markers of rheumatoid arthritis (RA). They include the so-called 'anti-keratin antibodies' (AKA) and anti-perinuclear factor (APF), and recognize human epidermal filaggrin and other (pro)filaggrin-related proteins of various epithelial tissues. In this study we demonstrate that AFA are produced in rheumatoid synovial joints. In 31 RA patients, AFA levels were assayed at equal IgG concentrations in paired synovial fluids (SF) and sera. AFA titre-like values determined by indirect immunofluorescence and immunoblotting and AFA concentrations determined by ELISA were non-significantly different in serum and SF, clearly indicating that AFA are not concentrated in SF. In contrast, we demonstrated that AFA are enriched in RA synovial membranes, since the ELISA-determined AFA in low ionic-strength extracts of synovial tissue from four RA patients represented a 7.5-fold higher proportion of total IgG than in paired sera. When small synovial tissue explants from RA patients were cultured for a period of 5 weeks, the profile of IgG and AFA released in the culture supernatants was first consistent with passive diffusion of the tissue-infiltrating IgG (including AFA) over the first day of culture, then with a de novo synthesis of IgG and AFA. Therefore, AFA-secreting plasma cells are present in the synovial tissue of RA patients and AFA can represent a significant proportion of the IgG secreted within the rheumatoid pannus.

[Gorham disease of the rib with osteolysis followed by bone remodeling. Study with magnetic resonance imaging]. / Maladie de Gorham costale avec ostéolyse puis réapparition osseuse. Etude en imagerie par résonance magnétique.

Gorham disease, or massive osteolysis, generally presents as osteolysis of a girdle bone. We report the case of a young adult who presented a fracture followed by destruction of the third right rib, then by regrowth eight months later.

Soft tissue tumors following traumatic injury: two observations of interest for the medicolegal causality.

Two cases of tumors of the soft tissues developing at the site of a previous traumatic injury occurring a few years earlier are reported. One was finally diagnosed as aggressive fibromatosis and the other as low-grade fibrosarcoma. Among the pathogenic mechanisms and the etiologic factors involved in such tumors, the posttraumatic causality is discussed, and in addition to the initial trauma, the role of iterative surgery in the first case and mineral muscular inclusions in the second case are examined. The different therapeutic approaches of such lesions are also reviewed.

Tumours and pseudotumorous lesions of the temporomandibular joint: a diagnostic challenge.

Tumours and pseudotumorous lesions originating from the synovial membrane of the temporomandibular joint are rare. We report a series of six cases of such disorders. There were two cases of synovial chondromatosis, two of calcium pyrophosphate dihydrate crystal deposition disease, one nodular synovitis and one synovial sarcoma. Three patients were female and three were male. Their ages ranged from 36 to 70 years. All had atypical clinical and radiographical presentation. The prevalence, clinical and radiographical findings and pathological features of each disease entity are discussed and a review of the literature is made concerning all tumours and pseudotumours arising from the temporomandibular joint.

[Systemic manifestations of primary Gougerot-Sjögren syndrome. Nature and incidence apropos of 34 cases]. / Manifestations systémiques du syndrome de Gougerot-Sjögren primaire. Nature et fréquence à propos de 34 cas.

OBJECTIVES: Assess the systemic manifestations observed in patients with primary Sjörgen's syndrome and the correlation between clinical findings and paraclinical data. METHODS: This retrospective study included patients who met the preliminary European criteria established in 1993. RESULTS: Among the 34 patients with primary Sjögren's syndrome (32 women, 2 men, mean age 54.9 years), systemic manifestations were observed as the inaugural sign in 25 (73.5%) and 31 cases (91%) during a mean 43-month follow-up. Clinical signs in 23 patients were: neuropsychiatric disorders including depressive syndromes (n = 17) and peripheral neuropathies (n = 13), joint and muscle disorders (n = 16), Raynaud's phenomenon (n = 8), pulmonary disorders (n = 8), digestive disorders (n = 8) including two cases of chronic autoimmune hepatitis, skin disease (n = 7), and renal (n = 3) and thyroid (n = 8) disorders. Antinuclear antibodies were found in 20 cases and anti-SSA antibodies in 5 associated with anti-SSB in 4. Eight patients had hematologic disorders. There was a correlation between the number of systemic signs (at least 2) and positive Rose-Bengal test (p = 0.045). DISCUSSION: Different systemic manifestations were found at percentages comparable with data in the literature except for Raynaud's syndrome which was less frequent and neurological disorders which were more frequent.

[Malignant granular cell tumor. Report of a clinico-pathologic case]. / La tumeur à cellules granuleuses maligne. A propos d'une observation anatomo-clinique.

The authors report a case of malignant granular cell tumor present in right buttock of a 40 year-old male. The diagnosis of these very rare (less than 50 cases reported in literature) and poor prognosis tumors is difficult. The diagnosis criteria of malignancy are reviewed regarding this clinical case and the literature.

[Fibrous dysplasia associated with intramuscular myxoma]. / Dysplasie fibreuse associée à un myxome des parties molles.

PURPOSE OF THE STUDY: The association of fibrous dysplasia and of soft tissue myxoma is rare. The authors report a new case and discuss the underlying mechanism. CASE REPORT: A 45 years male consulted for a large painful swelling of the right gluteal muscle. The CT scan revealed the soft tissue tumor associated with a bone cyst of the right pelvic bone. The mass was removed at operation and tumoral bone tissue was curetted, there were no evidence of tissue continuity between the two lesions. The histological diagnosis was intra muscular myxoma and fibrous dysplasia. DISCUSSION: Intra muscular myxoma associated to fibrous dysplasia of bone is a rare syndrome described by Mazabraud in 1957. A review of the literature until 1995 reveals that only 17 cases of this syndrome have been reported. Intra muscular myxoma is a rare benign soft tissue tumour arising from fibroblast. Fibrous dysplasia is a hamartomatous bony disorder, which appears in mono or polyostotic forms, with or without extraskeletal manifestation. CONCLUSION: It was not possible to demonstrate a clear correlation between the two lesions. The authors suggest that myxoma result from bone mechanical disorders.

[Adamantinoma and osteofibrous dysplasia of the tibia]. / Adamantinome du tibia et ostéo-fibrodysplasie.

PURPOSE OF THE STUDY: Fibrous dysplasia and adamantinoma of the long bones are two different entities encountered on the same part of the tibial shaft. The relationship between these two diseases seems to be established. We describe a case with some common, features arguing for a single pathology. MATERIAL AND METHODS: A seven year old boy presented in 1974 with a destructive lesion involving the cortex of the tibial shaft. The patient subsequently presented several fractures, treated surgically and has been followed for nineteen and a half years. The first biopsy specimen in 1974 led to the diagnosis of ossifying fibroma or fibrous dysplasia. It was reviewed with immunohistochemical technics but no epithelial component could be found. RESULTS AND DISCUSSION: These pathologic aspects led to the diagnosis of adamantinoma associated with osteofibrous dysplasia of the tibia. Twenty five such cases were found in the literature. Two theories are proposed: 1) these two diseases are independent and coexist on the same bone; 2) there is only one disease with different features that are either those of fibrous dysplasia or those of adamantinoma. Immunohistochemical technics are needed for diagnosis. CONCLUSION: Two sorts of adamantinoma can be observed on the long bones: adamantinoma without features of fibrous dysplasia with poor prognosis, adamantinoma with fibrous dysplasia's features called "differentiated adamantinoma" with a better prognosis.

[Neurologic and psychiatric manifestations of primary hyperparathyroidism. Study of 5 cases]. / Manifestations neurologiques et psychiatriques de l'hyperparathyroïdie primitive. Etude de 5 cas.

Among 8 of the 20 records of primary hyperparathyroidism examined in search of neurological and/or psychiatric manifestations, the authors extracted 5 clinical cases reported here. These 5 cases were selected because these manifestations had been well evaluated before and after curative surgery of the disease. Among the manifestations reported, there was one corpus striatum syndrome, one cervical myelopathy and three cases of "chronic" psychiatric disorders of several years duration completely cured after parathyroidectomy. The authors underline that such psychiatric disorders should be taken into account in the discussion of treatment.

[Adamantinoma of the tibia and osteofibrodysplasia. Report of a case]. / Adamantinome du tibia et ostéo-fibro-dysplasie. A propos d'une observation.

An adamantinoma of the tibia associated with morphologic aspects of osteofibrodysplasia is studied. The ipsilateral fibula has radiological lesions. The first diagnosis was osteofibrodysplasia and the adamantinoma was recognised eighteen years later. Twenty six cases of adamantinoma associated with osteofibrodysplasia have been published in the literature until 1991. The relationship between these two entities is emphasized. This kind of adamantinoma would have a better behaviour than the others.

[Painful polyarticular syndrome probably induced by cyclosporin in three patients with a kidney transplant and one with a heart transplant]. / Syndrome algique polyarticulaire probablement induit par la ciclosporine (SAPPIC) chez trois transplantes renaux et un transplanté cardiaque.

Among others, ciclosporin had the advantage of decreasing appreciably the incidence of osteonecrosis after renal transplantation. However, the authors have isolated, in three renal transplant patients and one heart transplant patient, treated with ciclosporin, a new syndrome consisting of bilateral and symmetrical joint pain, affecting the lower extremities and lasting almost one year. Subsiding of the radiological aspect and the isotopic hyperfixation of the affected joints, at the end of the evolution, as well as the histological aspect of the bone surrounding the joints, remind somehow of algodystrophy. In both cases, the severity of the pain seemed to depend on the doses of ciclosporin, indicating that this molecule could play a role in the occurrence of this syndrome.

[Psoriatic onycho-pachydermo-periostitis of the big toe. Anatomo-clinical study and physiopathogenic approach apropos of 4 cases]. / L'onycho-pachydermo-périostite psoriasique du gros orteil. Etude anatomo-clinique et approche physiopathogénique à propos de 4 observations.

The authors report 4 cases of rheumatoid psoriasis in which involvement of the great toe, associating psoriatic onychosis, thickening of the distal soft tissues and osteo-periostitis of the distal phalanx without lesions of the interphalangeal joint, is the result of a direct repercussion of the ungual lesion on the distal phalanx. This is explained by very close anatomic links between nail and distal phalanx. They advocate the term of psoriatic onycho-pachydermo-periostitis of the great toe or OP3GT, when referring to this radio-clinical syndrome highly evocative of rheumatoid psoriasis.

[Association of an adenoid cystic epithelioma with a clear cell carcinoma of the palate]. / Association d'un épithélioma adénoïde kystique et d'un carcinome à cellules claires du palais.

An infrequent polymorphous minor salivary gland tumor is described. It involved the palate and later invaded the upper jaw. The morphologic features are those of both adenoid cystic carcinoma and clear cell carcinoma. The clinical course, the cellular admixture of tumour components, and the immunohistologic results allow us to propose that clear cell carcinoma originating from adenoid cystic carcinoma offers the worst prognosis.

Chondromas of the hand. A report of thirty-five cases.

Twenty-nine patients were treated in the department of orthopedic surgery of Purpan (Toulouse), with thirty-five chondromata of the hand. Follow-up was over one year. Chondroma is a bone tumor predominantly of the left side, second ray of the hand and phalanx. Some multifocal cases have been observed. The diagnosis was made in half the cases through a pathological fracture. Chondroma is characterized by a diaphyseal or a metaphyso-diaphyseal lacuna, wearing away the cortical bone. Treatment consisted of curetting the tumor and filling the cavity with iliac cancellous bone graft. The clinical result was been excellent in 15 cases (60%), good in 6 cases (25%), with only some residual pain, and/or digital swelling. In two cases the finger was stiff, but functionally not disabling. In one case the stiffness was important and very disabling; after several recurrences it evolved into a chondrosarcoma.

[Subacute or initially chronic osteomyelitis in children. A series of 17 cases]. / L'ostéomyélite subaiguë ou chronique d'emblée chez l'enfant. Une série de 17 cas.

17 cases of subacute or at once chronic osteomyelitis are shown. At this occasion, an analysis of epidemiological, topographical, clinical and bacteriological data is given. An immunological study has been made in 5 cases: diagnosis is difficult at all stages. A reflexion is proposed concerning the treatment to be performed.