[Cerebral infarction, a complication of paroxysmal nocturnal haemoglobinuria]. / Infarctus cérébral complication de l'hémoglobinurie paroxystique nocturne.

Paroxysmal nocturnal hemoglobinuria (Marchiafava-Micheli disease) is a rare acquired clonal disorder of the hematopoietic stem cell. Its most frequent clinical manifestations are hemolytic crisis and the most serious are venous thrombosis of the mesenteric, hepatic, portal or cerebral territories. Arterial cerebral infarctions are extremely rare. We report and comment on a case of multiple strokes occurring during an hemolytic crisis and despite effective long-term anticoagulation treatment.

Subacute bacterial endocarditis presenting as polymyalgia rheumatica or giant cell arteritis.

OBJECTIVE: To report on several patients with subacute bacterial endocarditis who were initially presumed, incorrectly, to have polymyalgia rheumatica or giant cell arteritis. METHODS: We report 3 cases of subacute streptococcal endocarditis mimicking giant cell arteritis in 2 cases and polymyalgia rheumatica in one. We reviewed the literature through Medline search of French and English-language articles published between 1966 and 2005 and found 5 similar cases. RESULTS: Shoulder and/or pelvic girdle pain was associated with neck or back pain in all patients. Scalp tenderness, bilateral jaw pain, amaurosis fugax were present in 2 patients. One patient had no fever. Two patients were treated with corticosteroids with initial good clinical response in one. Appropriate antibiotic therapy resulted in the rapid disappearance of rheumatic complaints in 2 patients and achieved a definitive cure of endocarditis in all cases. CONCLUSION: Rheumatologic symptoms may hinder the correct diagnosis of infective endocarditis in patients who present with a clinical picture suggesting polymyalgia rheumatica or giant cell arteritis. In such cases, blood cultures should be systematically drawn.

Prognostic factors in polyarteritis nodosa and Churg-Strauss syndrome. A prospective study in 342 patients.

We undertook this study to determine the clinical, biologic, immunologic, and therapeutic factors associated with the prognoses of polyarteritis nodosa (PAN) and Churg-Strauss syndrome (CSS). Three hundred forty-two patients (260 with PAN, 82 with CSS) followed from 1980 to 1993 were included in a prospective study on prognostic factors. Two hundred eighty-eight of these patients were included in the prospective studies on PAN and CSS. Items to be considered for analysis were collected at the time of diagnosis, during the acute phase of the disease. A survival curve was plotted for each clinical and biologic symptom observed in PAN or CSS. Each treatment arm of the prospective therapeutic trials was also tested: 1) prednisone (CS) + oral cyclophosphamide (CYC) + plasma exchanges (PE) versus CS E, 2) CS + PE versus CS, 3) CS + oral CY versus CS + pulse CY, 4) CS + pulse CY + PE versus CS + pulse CY in severe PAN and CSS, and 5) PE + antiviral agents after short-term CS in hepatitis B virus-related PAN. Of the parameters thus evaluated, the following had significant prognostic value and were responsible for higher mortality: proteinuria > 1 g/d (p < 0.0001; relative risk [RR] 3.6), renal insufficiency with serum creatinine > 1.58 mg/DL (p < 0.02; RR 1.86), GI tract involvement (p < 0.008. RR 2.83 for surgery). Cardiomyopathy and CNS involvement were associated with a RR of mortality of 2.18 and 1.76, respectively; these were not statistically significant. Similar survival rates were obtained with the prospectively tested therapies. The five-factors score (FFS) we established considered the prognostic factors creatinemia, proteinuria, cardiomyopathy, GI tract involvement, and CNS signs. Multivariate analysis showed that proteinuria (due to vascular or glomerular disease) and GI tract involvement were independent prognostic factors. When FFS = 0 (none of the 5 prognostic factors present), mortality at 5 years was 11.9%; when FFS = 1 (1 of the 5 factors present), mortality was 25.9% (p < 0.005); when FFS > 2 (3 or more of the 5 factors present), mortality was 45.95% (p < 0.0001 between 0 and 2, p < 0.05 between 1 and 2). We conclude that an initial assessment of PAN or CSS severity enables outcome and mortality to be predicted. The FFS is a good predictor of death and can be used to help the clinician choose the most adequate treatment. Renal and GI signs are the most serious prognostic factors.

Churg-Strauss syndrome. Clinical study and long-term follow-up of 96 patients.

Churg-Strauss syndrome (CSS) is a systemic vasculitis characterized by the presence of asthma, hypereosinophilia, and necrotizing vasculitis with extravascular eosinophil granulomas. In this retrospective study of 96 patients between 1963 and 1995, we analyzed clinical manifestations, identified prognostic factors, and assessed the long-term outcome. CSS was diagnosed when asthma, hypereosinophilia > 1,500/mm3 or > 10%, and clinical manifestations consistent with systemic vasculitis, with or without histologic evidence, were present. Asthma was the most frequently observed manifestation at presentation, with mononeuritis multiplex the second. Other common manifestations were weight loss, fever, myalgia, skin involvement, paranasal sinusitis, arthralgia, pulmonary infiltrate, and gastrointestinal involvement. Mean eosinophilia at presentation was 7.193 +/- 6.706/mm3; ANCA, present in 20 of 42 (47.6%) patients, predominantly gave the perinuclear labeling pattern. All the patients were treated with corticosteroids alone or in combination with cyclophosphamide or plasma exchanges. Clinical remission was obtained in 91.5%; 22 (25.6%) patients relapsed. Twenty-three patients died during follow-up: 11 of these deaths were directly due to vasculitis. The presence of severe gastrointestinal tract or myocardial involvement was significantly associated with a poor clinical outcome. The long-term prognosis of CSS is good and does not differ from that of polyarteritis nodosa, although most patients need low doses of oral corticosteroids for persistent asthma, even many years after clinical recovery from vasculitis.

Polyarteritis nodosa related to hepatitis B virus. A prospective study with long-term observation of 41 patients.

Hepatitis B virus (HBV)-related polyarteritis nodosa (PAN) is a rare disease whose frequency has been decreasing over the past 10 years. We evaluated 41 patients with HBV-related PAN to determine the circumstances leading to infection, the clinical features of vasculitis, the prognostic factors, and the response to therapy. Most patients were first treated briefly with corticosteroids, and all were included in 2 nonrandomized prospective therapeutic trials of an antiviral agent (35 patients with vidarabine, 6 patients with interferon-alpha 2b) and plasma exchanges. The mean duration of follow-up was 69.6 +/- 44.8 months. At the end of the study, 21 (51.2%) patients had seroconverted to anti-HBeAb and 10 (24.4%) also had seroconverted to anti-HBsAb. In all, 23 (56%) patients no longer expressed serologic evidence of HBV replication. All 33 (80.5%) patients still alive at the end of follow-up recovered from PAN. Nineteen also recovered from HBV infection and were considered to be cured; 13 patients had persistent HBV infection and were considered to be in clinical recovery; and 1 patient was in remission, maintained with steroid therapy. Eight patients died during the study period; 3 deaths were directly attributable to PAN. HBV-related PAN is an acute disease, occurring shortly after infection and sharing the characteristics of classic PAN. It is not an antineutrophil cytoplasm antibodies (ANCA)-mediated vasculitis. The outcome was good for patients treated with short-term steroid therapy, antiviral agents, and plasma exchanges. We propose this protocol as the first treatment for HBV-related PAN, because it surpasses the conventional treatment with corticosteroids and cyclophosphamide, which facilitates viral replication and the development of chronic HBV infection.

Classification and management of necrotising vasculitides.

Systemic vasculitides are a heterogeneous group of diseases. Having only a partial understanding of the aetiologies and pathogenetic mechanisms of these disorders explains the difficulties encountered in classifying and treating patients. Nevertheless, some important points have been established. Classification is mainly based on the size of vessels affected and, from the polyarteritis nodosa group, microscopic polyangiitis (MPA) has been separated from classic polyarteritis nodosa (c-PAN). The latter is a rare disease which is, in a small number of cases, the consequence of hepatitis B or C virus (HBV/HCV) infection. In the other cases of c-PAN and in MPA, the aetiology is unknown as for Churg-Strauss syndrome (CSS) and Wegener's granulomatosis (WG). MPA, CSS and WG are mainly antineutrophil cytoplasmic antibodies (ANCA)-related vasculitides. ANCA play a part in the pathogenesis of diseases and are sometimes useful markers for diagnosis and follow-up. Vasculitis treatments should be chosen according to classification, aetiology, pathogenetic mechanisms, severity and predictable outcome. In virus-associated vasculitides, treatment is based on the combination of antiviral agents and symptomatic or immunomodulating therapies. HBV-related PAN and HCV-related cryoglobulinaemia respond to interferon-alpha and to plasma exchange. Responses are excellent in HBV-PAN but usually partial in HCV-cryoglobulinaemia, and relapses occur in the majority of cases. MPA, c-PAN, WG and other vasculitides respond to corticosteroids and cytotoxic agents, mainly cyclophosphamide. Treatment duration and ways of administration can vary from one disease to another. Plasma exchange is not recommended as the first-line treatment. Immunoglobulins and other immunomodulating treatments are indicated in limited cases and their indications necessitate further prospective studies.

Polyarteritis nodosa, microscopic polyangiitis, and Churg-Strauss syndrome. Clinical aspects and treatment.

In this article, three systemic vasculitides--polyarteritis nodosa, microscopic polyangiitis, and Churg-Strauss syndrome--are reviewed, focusing on advances in the understanding of the pathogenesis, new classification nomenclature, and major clinical features of these vasculitides. Distinguishing criteria between polyarteritis nodosa and microscopic polyangiitis are also presented in detail, and recent advances in the clinical management of these diseases according to appropriate therapeutic strategies are recommended.

Lymph node tuberculosis in the suburbs of Paris: 59 cases in adults not infected by the human immunodeficiency virus.

We report 59 cases of lymph node tuberculosis in adults not infected by the human immunodeficiency virus (HIV), observed over a period of 5 years in the North Eastern suburbs of Paris. There were 31 women and 28 men; 84.7% were aged under 44 years; 69.5% were not French, and 78% had exclusive lymph node tuberculosis. A superficial distribution was found in 52 cases and a deep pattern in 17 cases. Cervical and supraclavicular lymphadenopathies were the most common (64.4%). General symptoms were present in 63% of cases. The diagnosis was established by fine needle aspiration in 10 cases and by biopsy in 36 cases. Three cases of primary resistance to anti-tuberculosis therapy were described. Lymph node tuberculosis is still present in the Paris region, independently of HIV infection, probably due to poor social conditions.

Hepatitis C virus in patients with polyarteritis nodosa. Prevalence in 38 patients.

In order to assess the prevalence of hepatitis C virus (HCV) in polyarteritis nodosa (PN), 38 patients with systemic necrotizing angiitis were retrospectively tested for the presence of anti-HCV antibodies (Ab). Twenty-one patients were hepatitis B virus (HBV) positive, comprising group A, and 17 were HBV negative, comprising group B. Two patients from group A had anti-HCV Ab (2/21: 9.5%). One was treated unsuccessfully with corticosteroids, then with vidarabine and plasma exchanges; HBe/anti-HBe seroconversion was not observed and anti-HCV Ab disappeared 8 months after the onset of PN. The second patient was successfully treated with corticosteroids, then vidarabine and plasma exchanges; he recovered from PN, HBV seroconversion occurred, and the anti HCV Ab remained detectable. These results show that: 1) the prevalence of anti HCV Ab in PN related to HBV is nearly the same (9.5%) as the prevalence of HCV Ab observed in patients with chronic hepatitis related to HBV infection; 2) the course of these two viral infections can be different and the role of HCV as an etiologic factor in PN has not been established.

Persistence of antineutrophil cytoplasmic antibodies (ANCA) in asymptomatic patients with systemic polyarteritis nodosa or Churg-Strauss syndrome: follow-up of 53 patients.

OBJECTIVE: We retrospectively analyzed the significance of persistent ANCA positivity after clinical remission in 53 consecutive patients with histologically and/or angiographically proven polyarteritis nodosa (PAN), or Churg-Strauss syndrome (CSS) followed between 1981 and 1993. METHODS: ANCA were detected using an immunofluorescence assay and ELISA: Each patient met the American College of Rheumatology 1990 criteria for PAN or CSS. Clinical and biological evaluations were always essential factors in the decision to intensify therapy. RESULTS: ANCA were initially present in 15 patients (28.3%): 3/26 (11.5%) with HBV-related PAN, 6/18 (33.3%) with PAN of unknown etiology and 6/9 (66.7%) with CSS. Five patients remained ANCA-positive after clinical remission: 3 with PAN (one of them relapsed) and 2 with CSS who both relapsed. Among the 12 patients who died during follow-up, only 1 (8.3%) was initially ANCA-positive. Fifteen of the 41 survivors (29.2%) were ANCA-positive. CONCLUSION: Persistence of ANCA positivity in PAN and CSS may be a marker of an underlying disease process, but does not adequately reflect disease activity and, thus, in no case should be the only indication for therapeutic intensification.

Polyarteritis nodosa, microscopic polyangiitis, and Churg-Strauss syndrome: clinical aspects, neurologic manifestations, and treatment.

Polyarteritis nodosa (PAN), microscopic polyangiitis (MPA), and Churg-Strauss syndrome (CSS) all have neurologic symptoms and share characteristics and outcomes. Clinical aspects, neurologic manifestations, and treatment of these three diseases are examined in this article.

[Thrombo-aspiration in the surgical treatment of acute ischemia of the legs. Preliminary experience]. / La thrombo-aspiration dans le traitement chirurgical des ischémies aiguës des membres inférieurs. Expérience préliminaire.

Eleven thrombo aspirations were performed in ten patients with lower limb acute ischemia. Three were performed under local and 8 under general anesthesia. They were associated with 4 thrombectomies, 6 transluminal angioplasty procedures, 1 limb infusion, 1 treatment of a false aneurysm, 1 aponeurotomy, 1 angioscopy. Clinical and radiological success was achieved in 9 patients whereas there was one immediate failure requiring a popliteal distal bypass. Late partial rethrombosis requiring medical treatment occurred in one patient. Thrombo aspiration is a safe effective technique which can be combined with other endoluminal procedures in patients with acute lower limb ischemia.

[Meningoradiculitis disclosing T-lymphocyte leukemia in HTLV-1 infection]. / Méningo-radiculite révélatrice d'une leucémie à lymphocytes T au cours d'une infection à HTLV1.

A case of HILV1-associated adult T cell leukaemia/lymphoma (ATLL) in à 21-year olf African woman is reported. The patient presented with lymphomatous meningoradiculopathy. The usual clinical features of ATLL were absent. Lumbar MRI showed a pial enhancement by DTPA-gadolinium of the conus medullaris which extended to the proximal cauda equina. Under systemic chemotherapy coupled with intrathecal chemotherapy the patient progressively improved, and at the second MRI examination complete disappearance of the lumbar enhancement was observed. MRI of the brain using axial and coronal T2-weighted sequences detected multifocal lesions of high-intensity signal in the subcortical white matter. ATLL is unusual in people of African origin. The ATLL-strongyloïdes infestation association has previously been reported, suggesting that parasitic infestation may be an important co-factor leading to the development of ATLL.

[Allergic angiitis with granulomatosis: the Churg and Strauss syndrome]. / L'angéite allergique avec granulomatose: le syndrome de Churg et Strauss.

Churg-Strauss Syndrome is a disorder characterized by pulmonary and systemic necrotizing vasculitis, extravascular granulomas, and eosinophilia occurring almost exclusively in patients with asthma or a history of allergy. The clinical manifestations most commonly associated with asthma are mononeuritis multiplex, gastrointestinal, cutaneous, cardiac and renal involvement. ANCA are found in 2/3 of the patients with Churg-Strauss Syndrome and are usually p-ANCA. The treatment of Churg-Strauss Syndrome relies on corticosteroids and cyclophosphamide.

[Prevention of opportunistic infections in HIV seropositive patients. Prevention of parasitic infections]. / Prévention des infections opportunistes chez le sujet VIH+. Prévention des infections parasitaires.

PNEUMOCYSTIS CARINII PNEUMONIA: Below a threshold 200 CD4 cont, the risk of P. carinii infection is sufficiently high to propose systematic prophylaxis. Trimethoprim-sulfamethoxazole is the treatment of choice for primary and secondary prevention. Pentamidine-isothionate can also be used, possibly with dapsone. TOXOPLASMOSIS: There is a consensus on primary prevention in patients with positive toxoplasmosis serology whose CD4 count is under 100. Trimethoprime-sulfamethoxazole is the first intention drug, but poor tolerance may require conversion to dapsone-pyrimethamine. For secondary prophylaxis, the consensus is for life-long treatment with low-doses after initiating treatment with sulfadiazine and pyrimethamine. LEISHMANIASIS: Secondary prevention is reasonable using monthly infusions of pentamidine.

[Prevention of opportunistic infections in HIV seropositive patients. Prevention of bacterial infections]. / Prévention des infections opportunistes chez le sujet VIH+. Prévention des infections bactériennes.

TUBERCULOSIS: Primary prophylaxis is indicated in subjects at risks, i.e. those with a positive tuberculine test or a negative test and major immunodepression. BCG vaccine is definitely contraindicated in case of AIDS. Prescription of isoniazid in combination with vitamin B6 has been found to be effective. MYCOBACTERIUM AVIUM INTRACELLULARE: Prophylaxis concerns highly immunodepressed patients and is based on rifabutine. Prophylaxis is contraindicated in case of active tuberculosis. OTHER GERMS: Preventive care in food intake for potential digestive tract infections is required as well as antipneumococcal vaccine. Secondary prophylaxis is based on continuous antibiotics. The positive effect of intravenous immunoglobulins, especially in children, remains a question of debate.

[Prevention of opportunistic infections in HIV seropositive patients. Prevention of viral infections and mycoses]. / Prévention des infections opportunistes chez le sujet VIH+. Prévention des infections virales et des mycoses.

CYTOMEGALOVIRUS INFECTION: Primary prophylaxis is logical in high-risk subjects (CD4 < 50) who are asymptomatic; per os ganciclovir is under evaluation. Systematic secondary prophylaxis relies on intravenous ganciclovir or foscarnet. HERPES SIMPLEX AND ZOSTER: Secondary prevention with acyclovir is not recommended due to the risk of resistance. Herpes zoster retinitis is an absolute indication for continuous oral treatment with acyclovir. CRYPTOCOCCUS: Oral fluconazol is required for secondary prophylaxis. CANDIDIASIS: Due to the risk of resistant strains, neither primary nor secondary prophylasis is recommended. HISTIOPLASMOSIS: Risk of recurrence justifies secondary prophylaxis with itraconazole or fluconazole. COCCIDIODOMYCOSIS: Secondary prophylaxis with amphotericin B or fluconazole is mandatory. ASPERGILLOSIS: Itraconazole is the best agent when prophylaxis is needed.

[Pulmonary hyalinizing granuloma. Apropos of 2 new cases]. / Les granulomes hyalinisants pulmonaires. A propos de 2 nouveaux cas.

Pulmonary hyalinising granuloma are nodular or localised fibrosing lesions of the pulmonary parenchyma and are single or multiple. We report two new cases of this disorder which is rare, as only 62 cases have been published in the literature. It is a pathology with few symptoms, sometimes revealed by general signs. Radiologically there are nodules which are most often round or oval, intra-parenchymal, well demarcated, single or multiple. The histological appearance is characteristic: the centre of the granuloma consists of a network of dense collagen fibres which are lamellar separated by clear spaces; the periphery is the seat of rich cellular infiltration of plasmocytes and lymphocytes in the peri-vascular region. Most often there is a spontaneous benign outcome. The frequent association of pulmonary hyalinising granuloma in fibrotic disorders and the similarity in the histological appearance leads to the hypothesis of a common pathogenesis in these disorders.

[Synchronization of plasma exchange and cyclophosphamide in severe systemic diseases. A consecutive study of 10 patients]. / Synchronisation d'échanges plasmatiques et de cyclophosphamide dans les maladies systémiques sévères. Etude consécutive de 10 malades.

Ten patients with severe systemic diseases, including systemic lupus erythematosus (n = 2), polymyositis (n = 2), essential mixed cryoglobulinaemia (n = 2), rheumatoid arthritis vasculitis (n = 3) and Wegener's granulomatosis (n = 1), were treated with 3 consecutive plasma exchanges synchronized with pulse cyclophosphamide. This therapeutic regimen was applied every 4 weeks initially and thereafter every 6 weeks in case of positive response after the first 3 cycles; it was combined in all patients with corticosteroid therapy. The treatment was administered for severe flare-up of the disease in 7 patients and for failure of previous treatments, including corticosteroids, cyclophosphamide and plasmapheresis, in 3 patients. Three kinds of response were observed: lasting complete remission without relapse after synchronization had ceased in 4 patients, partial clinical remission with post-synchronization relapse in 5 patients, and primary failure without any clinical response to treatment in only 1 patient. These results suggest that repeated plasma exchanges synchronized with cyclophosphamide are effective against progressive autoimmune diseases and in cases where conventionally administered immunosuppressive treatments had failed. However, this type of treatment cannot prevent long-term relapses, and only a prospective study can evaluate its success in terms of survival.