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M F Kahn

Publications and source records attributed to M F Kahn.

At least 73 records · Page 4Linked to original sources
PubMed1996

[Systemic scleroderma and sarcoidosis: 3 new cases].

We observed 3 patients with successive scleroderma (SS) and (what is considered to be) sarcoidosis (SA). The diagnosis SS included in the 3 patients: Raynaud's syndrome with pulpal necrosis and capillaritis, sclerodactylia and acro-osteolysis, multiple joint pain and FAN+. Also observed were: esophagus involvement (n = 3), pulmonary artery hypertension (n = 1), telangiectasia (n = 2) and anti-Scl 70 (n = 2). Initially, all patients had restrictive pulmonary disease. SS was diagnosed 5 to 9 years prior to SA in 2 patients. Diagnosis of SA was based on the following arguments: Loëfgren's syndrome with erythema nodosa (n = 1), parotiditis (n = 2), sicca syndrome (n = 2), myalgia (n = 2), joint involvement (n = 2), non-infectious pluropericarditis (n = 2), epitheloid and giant cell granulomas without caseous necrosis (lung = 3, liver = 1, lymph nodes = 1, salivary glands = 1, synovia = 1), negative search for bacilli, elevated conversion enzyme (n = 1) and, in each case, by the lack of any other cause. One patient died from lung cancer and another from respiratory failure. Nome of the patients had primary biliary cirhosis. This rare association between SS and SA demonstrates the confluent limits of certain systemic diseases and raises a difficult problem to differentiate pulmonary involvement in these diseases. The gravity of this localization and the poor sensitivy to corticosteroids.

Adult↗
PubMed1995

[Destructive arthritis of the hip in Crohn disease. 10 cases].

OBJECTIVES: Destructive arthritis of the hip joint is a rare manifestation of Crohn's disease. We evaluated its frequency and clinical features in comparison with spondylarthritis and assessed the clinical course. METHODS: One hundred patients with Crohn's disease were followed prospectively in search of destructive arthritis of the hip joint. Patients with both Crohn's disease and manifestations of inflammatory joint disease hospitalized in three rheumatology units were also evaluated retrospectively. RESULTS: The prevalence of destructive hip disease was 2% in patients with Crohn's disease. Ten patients had destructive arthritis of the hip. Three had ankylosing spondylitis, 5 had spondylarthritis not responding to the criteria of ankylosing spondylitis and 2 had no axial joint disease. In all patients, signs of hip joint disease were a narrowed intra-articular space, bone condensation, osteophytosis and features of joint destruction. Manifestations were similar to those in patients with hip joint disease related to idiopathic ankylosing spondylarthritis. The clinical course was poorly controlled by anti-inflammatory drugs or treatment of the inflammatory bowel disease. Synoviorthesis were required and were effective in most cases. Total hip replacement was required in 3 cases. CONCLUSION: Destructive arthritis of the hip in patients with Crohn's disease is a rare complication causing severe functional impairment further handicapping the patient with a chronic bowel disease.

Adolescent↗
PubMed1995

[Current status of the SAPHO syndrome].

In 1987, our group proposed the acronym SAPHO (Synovitis Acne Pustulosis Hyperostosis Osteitis) to identify a clinical entity with characteristic manifestations formerly described under a wide variety of names. Since our first description, several new points have been recognized. For example it has been demonstrated that the skin lesions are not necessarily concomitant with osteoarticular manifestations sometimes separated by a long time interval. There are also several arguments suggesting a relationship with spondyloarthropathy. In addition, pseudo-tumoral fibrosis of anterior thoracic lesions which may lead to venous compression have been observed. It is difficult to determine the frequency of SAPHO. Most of the cases reported were observed in western Europe or Japan. Now that the concept has been accepted by our British colleagues, the number of cases reported has increased steadily, perhaps suggesting a reason for the apparently low frequency in the United States. The pathogenesis is still unknown. There is some evidence however that Propionibacterium acnes infection may be involved, at least in some cases, but formal proof is still lacking. Treating patients with SAPHO thus remains a question of debate. Non-steroid antiinflammatory agents are currently the treatment of choice although certain authors have tried colchicine and sulfasalazine or methotrexate with little success. There remains a good deal of work to be done before we thoroughly understand the SAPHO syndrome but we can nevertheless assure our patients that this strange bone disease is neither a tumour nor an infection and is never severely invalidating.

Acne Vulgaris↗
PubMed1995

Absence of correlation between IL-1 alpha intron 6 polymorphism and rheumatoid arthritis.

Several studies have implicated interleukin 1 alpha (IL-1 alpha) in the pathogenesis of rheumatoid arthritis (RA). We analysed IL-1 alpha intron 6 polymorphism in relation to RA (50 patients with RA and 50 healthy controls). The study of a healthy control population confirmed the existence of the different alleles with a frequency similar to that in the Caucasian populations of northern England. Allele and genotype distributions did not differ significantly between the normal and RA populations, although the allele corresponding to 8 repeats was over-represented in the RA population (8 and 14% in the healthy and RA populations respectively). This suggests that IL-1 alpha intron 6 polymorphism could be part of a complex process involving other unidentified genetic factors in the pathogenesis of RA.

Alleles↗
PubMed1995

Low-dose methotrexate: an effective corticosteroid-sparing agent in the musculoskeletal manifestations of sarcoidosis.

Extrapulmonary sarcoidosis, and particularly the presence of musculoskeletal complications of the disease, may require chronic corticosteroid therapy. In five patients with biopsy-proven sarcoidosis and presenting recalcitrant forms of the disease, we introduced low-dose oral methotrexate (MTX) [10 mg/week (7.5-15)] for 30 months (16-34) to control the clinical and biological symptoms as well as to try to reduce the intolerated steroid posology. Beneficial effects were observed within 8-12 weeks in all patients, which allowed a reduction of 59% (35-75) of the corticosteroid posology, and maintained with a follow-up of 3 yr in 4/5 patients. No significant toxicity was observed. MTX appears to be an efficient, safe and corticosteroid-sparing therapeutic agent for the treatment of recalcitrant musculoskeletal manifestations of sarcoidosis.

Administration, Oral↗
PubMed1995

Antibodies to small ribonucleoprotein and to 73-kD heat shock protein: two distinct markers of mixed connective tissue disease.

We set out to discover whether antibodies to small ribonucleoprotein antigens (RNP) and to 73-kD heat shock protein (hsp 73) which have been proposed as markers of mixed connective tissue disease (MCTD) recognize different epitopes. MCTD serum was immunoadsorbed on hsp 73-coupled Sepharose and the affinity retained, and non-retained fractions were checked by immunoblotting for recognition of either purified bovine hsp 73 or calf thymus extract RNPs. The hsp 73 affinity-bound serum fraction recognized hsp 73 but not RNP antigens, the reverse being true for the non-retained fraction. We conclude that anti-hsp 73 and anti-RNPs are distinct markers of MCTD.

Antigens, Nuclear↗
PubMed1995

Angiotropic large cell lymphoma with mononeuritis multiplex mimicking systemic vasculitis.

Angiotropic large cell lymphoma (ALCL), the so-called malignant angioendotheliomatosis, is characterised by proliferation of tumorous cells within small vessels. Manifestations in the CNS and cutaneous lesions prevail in the clinical presentation, although any organ can be involved. The recent classification of this lymphoma as part of the large cell lymphomas has modified the therapeutic approaches employed. This should improve the prognosis of this usually fatal disease. An unusual case presenting with fever, mononeuritis multiplex, and cutaneous lesions is reported. Peripheral neuropathy without other neurological symptoms is uncommon, and, to our knowledge, such isolated mononeuritis multiplex with nerve lesions has not been previously reported in ALCL. The clinical diagnosis was a systemic necrotising vasculitis and it is considered that its differential diagnosis must include angiotropic large cell lymphoma.

Aged↗
PubMed1995

SAPHO syndrome.

Explore the source record for details and available documents.

Acne Vulgaris↗
PubMed1995

Primary systemic Sjögren syndrome (SS) preceding systemic lupus erythematosus: a retrospective study of 4 cases in a cohort of 55 SS patients.

That SS and SLE can coexist is well established. The time relationship between the two diseases is however not well known. To determine whether SS can precede SLE, we reviewed the charts of 55 patients with SS diagnosed in our department between 1975 and 1985. All 55 patients fulfilled at least four of the six preliminary criteria for SS developed by the European Study Group for SS. At entry, none met four ARA criteria for SLE and none had anti-double-stranded DNA or anti-Sm antibodies. The mean follow-up was 12 years (range 8-18 years). Four of the 55 patients developed SLE (7.5%). High titres of anti-native DNA antibodies (> 35 IU RIA, normal < 7 IU) appeared in four patients (2-11 years after the diagnosis of SS). Two of these patients fulfilled at least four ARA criteria for SLE; two others had only three criteria. Two additional patients developed anti-Sm antibodies without anti-native DNA antibodies 1 and 10 years after the initial diagnosis of SS, respectively. Both patients fulfilled six ARA criteria for SLE. At entry, anti-SSA and/or anti-SSB antibodies were present in all six SS patients and persisted subsequently. The main clinical events suggesting progression to SLE were pleuropericarditis, glomerulonephritis and focal central nervous system disease. In conclusion, SS with extraglandular components can antedate SLE by many years. Anti-native-DNA and anti-Sm antibodies should be measured when atypical clinical manifestations occur in a patient with systemic SS.

Adult↗
PubMed1995

Primary Sjögren's syndrome (SS) and malignant lymphoma. A retrospective cohort study of 55 patients with SS.

The aim of this study was to assess the prevalence of malignant lymphomas in patients with long-standing primary Sjögren's syndrome (pSS). We retrospectively studied a cohort of 55 patients with pSS over a mean follow-up period of 12 years. Five patients (9%) developed malignant lymphoma. The interval between the diagnoses of SS and lymphoma ranged from four to 12 years (mean = 6.5 years). The lymphoma arose in the lymph nodes in two cases, the parotid gland in one case, the lacrimal gland in one case, and the lung in one case. All five cases were B-cell low-grade lymphomas. Among our SS patients, those with extraglandular manifestations and/or a mixed cryoglobulin were at increased risk for lymphoma development. Secondary lymphoma carried a poor prognosis in our study. Three of the six SS patients who died during the follow-up period had lymphoma.

Adult↗
PubMed1995

Familial multiple myeloma. Report of a case in a father and daughter. Review of the literature.

We report a case of familial multiple myeloma in a father and daughter. The same lambda chain was detectable in both patients. Symptom onset occurred three years earlier in the daughter than in the father. We found 52 previously published cases of familial myeloma, of which only 13 occurred in a parent and child. Given the incidence of myeloma in the general population, the occurrence of several cases in the same family is probably not due to chance. The few available data on karyotypes and oncogenes are discordant. The respective role of genetic factors and environmental factors is discussed.

Animals↗
PubMed1995

Ossification of the posterior longitudinal ligament, diffuse, idiopathic skeletal hyperostosis, abnormal retinol and retinol binding protein: a familial observation.

We describe a 52-year-old man who presented with diffuse idiopathic skeletal hyperostosis, ossification of the posterior longitudinal ligament, and abnormal levels of retinol and retinol binding protein (RBP). The molar retinol/retinol binding protein ratio was high, suggesting congenital functional RBP deficiency. His two sons, aged 23 and 27 years, shared the same biological abnormality without clinical symptoms. To our knowledge, this is the first case report of such a familial association.

Humans↗
PubMed1995

[Lymphomas of the ocular adnexa in Gougerot-Sjögren syndrome. Apropos of 4 cases].

The risk of malignant B cell lymphoma is increased in Sjögren's syndrome (SS). Orbital localization seems infrequent. We report 4 cases of malignant lymphoma (ML) occurring in 4 women aged 47 to 77 years, with primary SS in 3 cases, located to the conjunctiva in 2 cases, the lacrymal gland in 1 case and the eyelid in 1 case. The interval between the diagnosis of SS and orbital ML varied from 6 months to 15 years. All 4 lymphomas were of the B cell type, low histopathologic grade, with monoclonal gammopathy in 1 case. Extraocular lymphoma was initially present in 1 case. ML remained localized in 2 cases with a follow-up of 4 and 6 years. Two patients treated by excisional biopsy alone are in complete remission 3 and 6 years later. The 2 other patients treated with orbital radiotherapy and chemotherapy died rapidly (transformation into a high grade malignancy in 1 case). We conclude that clinical, immunopathologic features, as well as prognosis and treatment of ocular adnexa ML in SS are similar to those of primary ML without SS.

Aged↗