Two familial cases of malignant Reiter's syndrome.

A Cambodian man and his son concomitantly developed malignant Reiter's syndrome soon after their arrival in France. In both cases generalized skin lesions of pustular psoriasis and systemic features were present. The son died after 2 years of unresponsive continuously progressive disease. The father received pulses of high dose immunosuppressants that worked rapidly and prevented a life threatening course. These 2 cases illustrate the pathophysiologic hypothesis of Reiter's syndrome, emphasizing the role of environmental triggering factors and the relationship between spondylarthropathies in B27 positive patients. Fatal cases of Reiter's syndrome are very rare in the review of the literature.

Sjögren's syndrome diagnosis: a comparison of conjunctival and gingival impressions and salivary gland biopsy.

This study reviewed 43 patients with Sjögren's syndrome. All of the patients complained of either dry eye and/or dry mouth. Their clinical records were compared with results of three laboratory diagnostic tests to determine the most sensitive test. Out of 30 patients who complained of both dry eye and dry mouth, 10 had positive conjunctival impressions, 20 had positive gingival impressions and 24 had positive salivary gland biopsies. Of 5 patients who suffered from only dry mouth, 2 had positive conjunctival impressions, 3 had positive gingival impressions and 3 had positive salivary gland biopsies. Of 7 patients with only dry eye complaints, there were 3 positive conjunctival impressions, 5 positive gingival impressions, and 6 positive salivary gland biopsies. Out of 6 healthy controls without complaints of dry eyes/mouth, there were no positive conjunctival impressions, 4 positive gingival impressions, and 5 positive salivary gland biopsies. Gingival impression appears to be nearly as sensitive as salivary gland biopsy in this group of patients suffering from Sjögren's Syndrome.

[Treatment with iloprost of critical ischemia of the lower limbs associated with cardiac insufficiency. Study of interaction with the pharmacokinetics of digoxin]. / Traitement par Iloprost des ischémies critiques des membres inférieurs associées à une insuffisance cardiaque. Etude de l'interaction avec la pharmacocinétique de la digoxine.

We have treated with intravenous iloprost twelve patients suffering from cardiac insufficiency compensated under oral digoxin (NYHA class II) associated with severe limb ischaemia due to arterial insufficiency. Our aim was to study its possible interaction on digoxin levels and to evaluate the long-term efficacy of iloprost. Although iloprost slowed the digoxin absorption by approximately one hour, we found no clinically significant difference between the digoxin pharmacokinetic data before and during treatment by iloprost. Moreover, 11 out of the 12 patients had a good clinical fate after the treatment, which persisted at 6 months. The pain disappeared in 4 and diminished in 7; and all skin ulcers healed. This improvement has lasted up to two and a half years in two patients. The clinical tolerance of iloprost was acceptable despite frequent headache and flushing associated with hypotension and nausea. We conclude that iloprost seems to be a very promising treatment of severe limb ischaemia when no intervention on the proximal arteries is possible. The patients on digoxin can continue their treatment without dose alteration while starting on iloprost.

[Breast prostheses and their complications]. / Les prothèses mammaires et leurs complications.

In the past few years silicone breast implants have been a highly charged and controversial topic for psychologic, health and politico-legal reasons for patients and for surgical, medical and research concern for professionals. Both sides are interested in knowing about the complications encountered with these implants on one hand and to find the ideal device needed for mammary reconstruction on the other. Indeed silicone does not fulfill the characteristics initially expected for a synthetic soft-tissue substitute. The local cellular response leads to the formation of a capsule around the implant with frequently unsatisfying cosmetic results. The well described leakage occurring through the silicone envelope allows the silicone gel to diffuse to multiple anatomic areas in the body. Scientific works demonstrated that silicone gel acts as a potent immunologic adjuvant and can induce antibodies to silicone surface-associated antigens. Breast implanted women who present with systemic clinical symptoms can be included either in the group of well defined connective tissue diseases or in the atypical connective tissue disease-like syndrome. The largest epidemiologic studies have shown reassuring evidence against large hazards but have documented a low but statistically significant risk of connective-tissue diseases in women with silicone gel filled implants. Practitioners must manage these patients with the awareness that reliable objective tests to identify the useful problem are not yet available.

[Recklinghausen's disease with hypophosphoremia and osteomalacia. Apropos of 2 cases]. / Maladie de Recklinghausen avec hypophosphorémie et ostéomalacie. A propos de 2 observations.

The authors report two cases of osteomalacia due to phosphate diabetes in patients with Recklinghausen's disease. In one case abnormalities of vitamin D metabolism were identical with those described in cases of phosphate diabetes associated with mesenchymal tumours. The abnormalities found in the second case were different. The physiopathological mechanism of osteomalacia due to phosphate diabetes in mesenchymal diseases generally and in Recklinghausen's disease in particular is discussed.

[Risk of nosocomial Pneumocystis carinii pneumonia in immunosuppressed patients non-infected by human immunodeficiency viruses]. / Le risque de pneumocystose nosocomiale chez les immunodéprimés non infectés par le virus de l'immunodéficience humaine (VIH).

We report four cases of Pneumocystis carinii pneumonia (PCP) in Human Immunodeficiency Virus (HIV)-seronegative patients. Two of them had been hospitalized for polymyositis treatment near AIDS patients, respectively 1 and 4 months before PCP. The two others suffered from localized cancer. Their evolution was complicated by respiratory distress and death in two of them. A telephone survey among 19 hospital units yielded nine cases of similar patients. They were only observed in wards caring for AIDS patients at the same time, thus raising the question of a possible nosocomial transmission of PCP between AIDS patients and immunocompromised HIV-seronegative patients. This adds to the growing concern for hospital-acquired infections, including resistant tuberculosis and other opportunistic pathogens. We propose some practical measures to limit this risk by simple means such as no-contact between at-risk populations, enhanced disinfection procedures of the rooms and masking of the coughing PCP patients.

[Disseminated intravascular coagulation associated with prostatic cancer]. / Coagulation intravasculaire disséminée associée au cancer de la prostate.

Disseminated intravascular coagulation (DIC) associated with prostate adenocarcinoma is a bad prognostic sign. Most of the cases are limited to biological abnormalities. Some, however, come to medical attention due to thromboembolic or hemorrhagic complications. We report 4 such cases and review the pertinent literature. The characteristic features are low platelets and coagulation factors in an elderly man. In two out of the four cases, bleeding due to the DIC revealed the cancer. All patients received hormonotherapy and heparin. The worst fate (case 3) was a subacute one with no effect of the drugs and death in a short time. The other cases went into a five- to seven months remission before uncontrollable bleeding led to death. No favorable effect of the chemotherapy was observed. Thus, new treatments are sought for this rare but ominous complication of prostate cancer.

[Treatment with iloprost of critical ischemia of the lower limbs associated with cardiac insufficiency. Study of the interaction with pharmacokinetics of digoxin]. / Traitement par iloprost des ischémies critiques des membres inférieurs associées à une insuffisance cardiaque. Etude de l'interaction avec la pharmacocinétique de la digoxine.

Twelve patients with critical ischaemia of the lower limbs were treated with iloprost. The purpose of this study was to investigate for a possible iloprost-digoxin interaction and to evaluate the clinical benefit provided by short- or long-term iloprost therapy. The pharmacokinetics of digoxin were studied before and during iloprost treatment. Under iloprost the absorption of digoxin was delayed by about one hour, but the area under the plasma digoxin concentration curve remained unmodified. In 11 of our 12 patients the clinical effect of iloprost was satisfactory both immediately and after 6 months. Pain vanished in 6 patients and diminished in 6 patients. All skin ulcers were healed. In most cases this improvement persisted beyond the study period: 2 patients treated at the beginning of the study and who are still followed up have remained improved after 2 1/2 years. Two patients with pain relapse received iloprost in repeated 10 days' courses with successful results. The treatment was relatively well tolerated (headaches, flushing, abdominal pain). Thus, iloprost can avoid amputation in severe arteritis unsuitable for revascularization and for which there is no effective treatment. Patients under digoxin may continue to take this drug in the same doses during treatment with iloprost.

[Periarteritis nodosa with heart valve disease disclosed by occlusion of the central artery of the retina]. / Périartérite noueuse avec atteinte valvulaire cardiaque révélée par une occlusion de l'artère centrale de la rétine.

We report a case of periarteritis nodosa revealed by blindness due to occlusion of the central retinal artery. The disease appeared after a serous otitis and was accompanied by thrombocytosis. Its outcome was favourable under corticosteroid therapy, but it was complicated by aortic valve regurgitation. Lesions of fibrinoid necrosis were found on the aortic cusps removed during surgical replacement. It seems highly probable that the ocular and valvular lesions were specific, although this cannot be directly demonstrated. The case presented here is exceptional, and the relevant literature is reviewed.

[Psychiatric manifestations of lupus erythematosus systemic and Sjogren's syndrome]. / Troubles psychiatriques du lupus érythémateux disséminé et du syndrome de Gougerot-Sjögren. A propos d'une observation d'un syndrome de Gougerot-Sjögren secondaire associé à un lupus érythémateux disséminé.

UNLABELLED: We present one case of Sjögren's syndrome (SS) secondary to systemic lupus erythematosus (SLE) with predominant psychiatric manifestations, treated with success by cyclophosphamide. From this case, we review the psychiatric aspects of these two autoimmune diseases as described in the literature and we present the etiopathogenic hypothesis and treatment of the psychiatric disorders. Case report--In August 1996, a 38 year old man was admitted in our psychiatric department for agitation. Primary SS had been diagnosed in July 1996. He had previously attempted to suicide but was never hospitalized in a psychiatric department. During the hospitalization in our department, the patient had auditive hallucinations and felt persecuted. He received loxapine 400 mg/day and was remitted in a few days. He was discharged to a convalescent home with the diagnosis of brief psychotic disorder. In October 1996, he was readmitted to our department for agitation. He had shown agitated behavior and aggression in the convalescent home. There were no hallucinations and no affective disorders. He became calm rapidly and was discharged home a few days later. In November 1996, he was found in a coma by a neighbor. He was admitted to an intensive care unit. The lumbar punction revealed blood cells. Cerebral computer tomography showed subarachnoid hemorrhage. The diagnosis was meningeal hemorrhage due to vasculitis. After regaining consciousness, the patient complained of reduced visual acuity. This was believed to be due to retrobulbar neuritis and the patient's vision improved slightly with corticosteroids. The third hospitalization in our department occurred in February 1997 for depression. The patient had shut himself away for days in his apartment. He had suicidal ideas. His mood improved progressively under fluoxetine 40 mg/day. He was discharged to a convalescent home with the diagnosis of major depressive disorder. The fourth and last admission in our department occurred in June 1997. There were disturbances of memory and orientation. He felt sad and guilty about accusation of sexual abuse on his daughter. He presented typical histrionic symptoms: he had catatonic attitudes only in public areas such as the corridors. Cerebral computer tomography and electroencephalogram were normal. There was no biological abnormality. Signs of confusion rapidly disappeared. He felt better after reintroduction of fluoxetine 40 mg/day. Diagnosis was non-specified depressive disorder, but this episode could be retrospectively seen as delirium. After being hospitalized on these four occasions in one year in our psychiatric department, the diagnosis of his systemic disease was revised by rheumatologists. The patient was diagnosed as suffering from systemic lupus erythematosus associated with secondary Sjögren's syndrome. From September 1997, he received cyclophosphamide 2 g intraveinously per month during 6 months. His vision improved dramatically. His ocular dryness became milder. His mood is now stable. He has not suffered from hallucinations or delusion since. Psychiatric disorders in SLE--During the course of SLE, the occurrence of psychiatric manifestations varies widely from 5 to 83%. They include psychotic disorders, major depressive disorders, subtle cognitive disorders and personality disorders of histrionic type. Etiopathogenic hypothesis are: direct activity of the disease on the central nervous system by autoantibodies (antiphospholipide and antiribosome P autoantibodies) (18, 19) or cytokines (interleukin 2, interleukin 6, alpha interferon) (38, 59), side-effects of glucocorticosteroids and hydroxychloroquine (16) or anxious reaction to a chronic and potentially lethal illness (43, 54). Nevertheless, immunologic and cerebral imagery research suggests that psychiatric disorders are related to vasculitis and non-inflammatory vasculopathy of the small cerebral blood vessels. The management of the patients should include treatment of the disease itself and specific psychotropic treatment. Glucocorticosteroids and especially intravenous infusions of immunosuppressive agents, such as cyclophosphamide, are effective. Psychotropic drugs must be used, making sure to avoid SLE-inducing drugs, like chlorpromazine, carbamazepine and lithium carbonate (19, 20, 45). In addition, psychologic care is essential. Psychiatric disorders in SS--During the course of the primary SS, the occurrence of psychiatric disorders is large as well: from 20 to 70% (47, 61, 62). They are mainly major depressive disorders, anxiety disorders, cognitive disorders and dementia. Brief psychotic disorders and delirium are rare. Etiopathogenic hypotheses are similar as those in SLE, with some differences: antiphospholipide and antiribosome P autoantibodies are not usually found in SS and anti-Ro (SSA) autoantibodies in serum are associated with psychiatric disorders (3-11, 61). According to Drosos et al. (29, 30), psychiatric disorders are explained by psychological distress. This slowly progressive fluctuating disease creates constant discomfort from dysphagia, dyspareunia and functional disability. Some of these manifestations can be treated by corticosteroids and psychotropic drugs. Drugs with anticholinergic side-effects, like phenothiazines, tricyclic antidepressants and hydroxyzine which can enhance the oral dryness have to be avoided. Social and psychological support is important too. DISCUSSION: The diversity of psychiatric morbidity in SLE and SS may be due to differences in patient selection and a lack of uniform clinical criteria. Studies which use standardized diagnostic criteria and control groups don't allow one to come to a conclusion about the relative prevalence of the psychiatric disorders in these autoimmune diseases. This will probably be resolved thanks to the recently published "American College of Rheumatology nomenclature and case definitions for neuropsychiatric lupus syndromes" (1). Finally, we can ask ourselves if there is a significant number of undiagnosed SLE and SS in psychiatric departments. Two studies report systematic search for SLE in psychiatric patients. In 1992, Hopkinson et al. (39) searched for several autoantibodies in serum samples of nearly 300 hospitalized psychiatric patients. In 1993, Van Dam et al. (65) did the same with more than 2,000 patients admitted to a psychiatric hospital. Hopkinson et al. found 1% undiagnosed SLE, which is much higher than in general population, and recommended to search SLE in every patient with a high erythrocyte sedimentation rate in psychiatric services. Results of the Van Dam et al. study suggest on the contrary, that SLE is not a common cause of admission to psychiatric hospitals. There is no study which report systematic search of Sjögren's syndrome in a psychiatric department. This is probably because most of patients receive or have recently received psychotropics with anticholinergic side-effects which is an exclusion criteria of SS. CONCLUSION: Psychiatrists should keep in mind that SLE and primary SS are potential causes of psychiatric manifestations when examining patients with multiple unexplained somatic complaints and psychiatric symptoms. They should then search for autoantibodies in the serum after careful physical examination. Diagnosis of SLE or SS could lead to a better adapted prescription of corticosteroids and/or immunosuppressive drugs and specific psychotropic drugs, making sure to avoid lupus-inducing drugs in SLE and drugs with anticholinergic effects in SS. The existence of psychiatric manifestations in SLE and SS constitutes an indisputable clinical reality that each practitioner must be able to recognize and treat.

[Gougerot Sjögren syndrome and hepatitis C virus: what relation?]. / Syndrome de Gougerot Sjögren et virus de l'hépatite C: quels liens?

The incidence of Sjogrëns syndrome (SS) in patients with chronic liver disease and that of hepatitis C in mixed cryoglobulinemia strongly-suggest a link between the hepatitis C virus (HCV) and SS. A recent report has also demonstrated typical lymphocyte sialadenitis in HCV-positive subjects, raising a question concerning the classical definition of SS. Do patients with HCV and lymphocyte sialadenitis have SS? If the problem of variable diagnostic criteria can be overcome (for example by using the criteria established by the European study group) it can be concluded that the proportion of HCV+ patients with SS is related to female sex, age (perimenopause period in women), and liver histology rather than fibrosis, but not with duration of the liver disease, nor cirrhosis or viral genotype. The second question is to determine whether the observed focal lymphocyte infiltration of the salivary glands is typical of SS. As routine biopsy results lack specificity and sensitivity, immunohistochemistry is required to identify T8 predominance distinctive of SS. Results obtained to date suggest that the T8 sialadenitis might result from an autoimmune mechanism and consequently that the SS-HCV association might either be a coincidence between the two relatively frequent diseases or on the contrary that HCV plays a pathogenic role in SS. The major argument for the latter hypothesis would be the demonstration of HCV within the salivary gland epithelial cells. As HCV-positive immunohistochemistry tests on salivary biopsies may simply indicate presence of HCV in the blood stream at the time of biopsy, more sophisticated in situ PCR methods are currently being applied in an attempt to obtain objective evidence which could incriminate HCV infection of the salivary glands as a causal agent in Sjogrëns syndrome.

[Retrospective evaluation of an algorithm for the etiological diagnosis of hypercalcemia]. / Evaluation rétrospective d'un algorithme pour le diagnostic étiologique d'une hypercalcémie.

Aetiological diagnoses obtained by means of an algorithm were retrospectively compared with those made in the Internal Medicine Department of the La Pitié Hospital, Paris, in 22 cases of hypercalcaemia (24 causes detected). These cases covered all the usual causes of the disease. The algorithm produced no erroneous diagnosis. In 2 cases it would not have resulted in a precise diagnosis, but in all other patients the diagnosis obtained by this method was in agreement with the clinical diagnosis. Proposals are put forward for an improved version of the algorithm that would provide more rapid diagnoses and avoid the risk of a "no diagnosis" answer.

[Primary Gougerot-Sjogren syndrome. Spontaneous one-year course of clinical, biological and histological signs]. / Syndrome de Gougerot-Sjögren primaire. Evolution spontanée sur un an des signes cliniques, biologiques et histologiques.

OBJECTIVES: The natural clinical course of primary Sjögren's syndrome was followed in 8 patients to identify the concomitant functional, clinical, biological, scintigraphic and histological manifestations of the disease. METHODS: The diagnosis of primary Sjögren's syndrome was made on the basis of functional signs (ocular or salivary sicca syndrome) and 2 positive tests among the 3 objective ocular tests (Schirmer's test, break-up time, Rose Bengale). Work-up included recording of functional and clinical signs, ophthalmologic examination and laboratory tests at diagnosis and every 3 months for 12 months. Scintigraphy of the salivary glands was performed together with a biopsy at diagnosis and at 12 months. RESULTS: No one parameter varied significantly over a 1 year period demonstrating the lack of need for renewed examinations for diagnosis or regular follow-up. CONCLUSION: This is the first report providing a homogeneous series studied by one team over a determined period of time. It demonstrates that clinical, biological and anatomic criteria for primary Sjögren's syndrome do not show any correlation between functional signs and objective ocular tests.