[Subcutaneous calcifications in systemic lupus erythematosus].
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Biomedical subjects
Publications and source records attributed to M H Houman.
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Improved understanding of the pathogenic mechanisms of Behcet's disease (BD), and especially of the molecular basis involved in its pathogenesis, has sparked a new generation of potential BD treatments with improved side effect profiles and/or more specific targeting of the immune system. These therapies include new immunosuppressants, biologic medications, tolerizing agents, and immunoablation techniques, a number of which are currently in use in clinical practice (interferons). While some of these new therapies target specific inflammatory mechanisms in SLE (tumor necrosis factor-alpha inhibitors), others work by non-specific inhibition of the immune system (immunoablation). Each of these approaches will be discussed in this review.
Behçet's disease is a multi systemic vasculitis of unknown origin characterized by recurrent oral and genital ulcerations, uveitis and skin lesions. Vascular involvement mainly interesting the venous system is present in about 7 to 38% of patients. Arterial involvement is present in 2 to 8% of patients and is associated with a high mortality. It may present as aneurysms, pseudo aneurysms or arterial occlusions. Pseudo aneurysm formation has been reported to be sometimes due to invasive imaging techniques, it may also follow arterial repair. We report two exceptional localizations of iatrogenic pseudo aneurysms due to accidental arterial puncture, involving respectively the internal carotid artery and the humeral artery. We discuss the clinical characteristics and therapeutic modalities of this complication.
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INTRODUCTION: Extranodal involvement in sinus histiocytosis with massive lymphadenpathy (SHML) or Rosai-Dorfman disease is common, seen in 43% of cases. We present a case of disseminated form of SHML with thyroid, renal, bone and lung involvement. EXEGESIS: A 53-year-old woman presented with cervical lymphadenopathy and a large palpable mass in the submandibular area. A thyroid nodule was palpable. Laboratory data showed an increase of the erythrosedimentation rate and a polyclonal hypergammaglobulinemia. Histopathological examination of a cervical lymph node biopsy showed typical features of SHML. CT scan showed enlargement mediastinal lymph node, mass of the right lung and infiltrative mass in the right renal hilium. Radiographs revealed lytic lesions in the right proximal tibia and left patella. Histopathology of a biopsy from the kidney mass and thyroidectomy displayed typical characteristics of SMHL. The patient was treated by steroids with a markedly regression of the kidney and renal masses. CONCLUSION: Multiple extranodal involvement in SMHL is rare, the prognosis may be poor when lesions are massive and involve vital organs.
INTRODUCTION: The involvement of the peripheral nervous system in Behçet's disease is very rare. CASE REPORT: We report a case of a 47-year-old man with a six-year history of Behçet's disease and a two-year history of peripheral nervous system involvement. This patient presented with paraesthesia and weakness of the upper and lower limbs, diarrhea and erectile dysfunction. The electromyogram showed evidence of an axonal sensorimotor neuropathy and the nerve biopsy showed an axonal neuropathy. Routine blood tests were normal, there was no increase of serum creatine kinase, aspartate aminotransfease or lactate dehydrogenase and no signs of hyperthyroidism. Fibroscopy and colonoscopy showed no signs of entero-Behçet. The patient was treated with prednisone, cyclophosphamide and carbamazepine with an improvement of paraesthesia. CONCLUSION: The mechanism of the peripheral neuropathy in Behçet's disease is still unknown, it might be due to vasculitis of the vasa nervorum or to the side effects of colchicine. Our report is particular by the association of sensorimotor and autonomic involvement of peripheral neuropathy in a patient with Behçet's disease.
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Cardiac tIssue is a very rare localization for benign lipoma. Interseptal lipoma and lipomatous hypertrophy of the interatrial septum are two distinguished entities. We report the case of a 58-year-old patient who developed lipomatous hypertrophy of the interatrial septum which was revealed by arrhythmia. Diagnosis was suspected at magnetic resonance imaging and was confirmed intraoperatively and histologically.
Intracardiac thrombus formation is exceptional in Behçet's disease (less than 50 cases had been reported). Among 180 patients with Behçet's disease (according to the criteria of the international study group for Behçet's disease), three were diagnosed as having intracardiac thrombus. All were male, the mean age at the time of the diagnosis of the intracardiac thrombus was 27 years and the mean disease duration was 4.8 years. The presenting symptom was hemoptysis in two patients and heart failure in the third. Surgery was the first attempted treatment in two patients but thrombus recurred and resolved after medical treatment. The third patient was given corticosteroids and anticoagulants as the first line treatment. The thrombus did not resolve and the patient declined surgery. Intracardiac thrombus formation should be considered when a young Behçet's disease patient presents with an intracardiac mass. Medical treatment including corticosteroids, immunosuppressive drugs and anticoagulants should be considered as the first line treatment and surgery should be considered when there is no resolution of the thrombus or when it becomes massive and extensive. In certain cases, thrombolytic treatment becomes an interesting alternative to surgery.
There is a wide variation in the natural history of systemic lupus erythematosus (SLE) among different ethnic and geographical groups. Studies in Arabs are few and those in North Africans and especially in the Tunisian population do not exist. This study aims to demonstrate the demographic, clinical and laboratory characteristics of SLE Tunisian patients and to identify those at high risk for renal and neuropsychiatric involvements. One hundred patients with SLE (American College of Rheumatology criteria), seen at the Department of Internal Medicine of the University Hospital La Rabta in Tunisia over a 15-year period (1987 to 2001) were retrospectively enrolled. There were 92 women and eight men with an average age at the onset of disease of 32 years. Nineteen patients were aged over 50 years at the moment of SLE diagnosis (late-onset SLE). Of the patients, 78% had articular involvement, 53% photosensitivity and 63% malar rash. Serositis occurred in 45 patients of whom 16 had pericarditis and 29 had pleuritis. Nephritis was diagnosed in 43% of the cases and consisted always of glomerular nephritis, in three cases of which tubulointerstitial lesions were also observed. Comparison of patients with and without renal involvement showed that lupus nephritis was significantly associated with pericarditis (P = 0.03), arterial blood hypertension (P < 0.0001), cryoglobulinemia (P = 0.07) and antiphospholipid syndrome (P = 0.03). The SLEDAI at SLE diagnosis was significantly higher for lupus nephritis patients. Twelve patients with lupus nephritis died compared with three patients in the remaining group (P < 0.0001). Neuropsychiatric manifestations were observed in 25% of the cases. The mean age at SLE onset was significantly lower, the mean SLEDAI at SLE diagnosis and the mortality were significantly higher in the neuropsychiatric group than in the remaining group. Immunological features included antinuclear antibodies (100%), anti-DNA antibodies (56%), anti-Sm antibodies (61%), anticardiolipin antibodies (62%), anti-beta2GP1 (13%) anti-Rnp (23%) and hypocomplementemia (48%). The frequencies of pulmonary hypertension (25 versus 2%, P < 0.00001) and vascular thrombosis (25 versus 2%, P < 0.00001) were significantly higher in patients with positive anti beta2GP1 antibodies. The five-year survival rate in our series was 86%. The most frequent causes of death were active SLE and infections.
Pulmonary involvement in Behcet's disease is uncommon and pulmonary arterial aneurysms are present in about 1 to 7% of patients. But despite their scarcity, they represent a life-threatening complication and are usually regarded as of a poor prognosis. Our study aims to describe the epidemiological and therapeutic aspects of this condition. Among 180 patients with Behcet's disease according to the criteria of the international study group for Behcet's disease, four were diagnosed as having pulmonary artery aneurysms (2.22%). All were male, the mean age at the time of the diagnosis of pulmonary artery aneurysms was 29.8 years and the mean disease duration was 6.6 years. Hemoptysis was the presenting symptom in all cases. The patients were treated by corticosteroid, colchicine and cyclophosphamide pulses. The outcome was good with disappearance of hemoptysis in all cases and disappearance of pulmonary aneurysms in three. One patient had recurrence of aneurysms 40 months after the first episode and died 18 months later. So, we can hope, thanks to medical treatment, a better outcome than previously reported in this complication.
INTRODUCTION: The concomitant unexpected arrival of a breast tuberculosis and cerebral tuberculomas to a same immunocompetent patient has not been ever described in the literature. EXEGESIS: We report the case of an non-HIV-infected woman who presented a pulmonary, breast tuberculosis and intracranial tuberculomas. Investigation for acid-fast bacilli in sputum and cerebrospinal fluid was negative. The patient received an antituberculous therapy for 15 months, which led to the disappearance of the cerebral lesions. CONCLUSION: Our case was particular by the affected organs, the absence of immunodeficiency and the favorable outcome.
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OBJECTIVES: We present a case report of Rosai-Dorfman disease with multiple extranodal involvement and favorable outcome after steroid therapy. MATERIAL AND METHODS: Rosai-Dorfman disease was diagnosed in a 17-year-old man with orbital, lacrymal, and rhinopharyngeal localizations. Histopathologic examination of lymphadenopathy biopsy revealed the diagnostic of Rosai-Dorfman disease. We present the histological findings leading to diagnosis and radiological data. RESULTS: Conservative treatment with oral steroids resulted in the resolution of cervical lymphadenopathy and extranodal lesions. The patient was free of problems nine mouths after discontinuation of his treatment, demonstrating its efficacy and safety. CONCLUSION: Rosai-Dorfman disease is a benign disorder and steroid treatment must be initiated in cases with significant cosmetic deformity.
PURPOSE: To describe epidemiological and clinical characteristics of neurological involvement in Behçet's disease (BD) and to determine a subgroup of patients at high risk for this complication. PATIENTS AND METHODS: The medical notes of 105 patients with BD fulfilling the criteria of the international Study Group for Behçet's disease were retrospectively reviewed. Patients were divided into two groups according to the presence (group 1) or not (group 2) of neurological and/or psychiatric involvement attributable to BD. The epidemiological, clinical and genetic (HLA B51 and MICA 6 frequency) features in the two groups were analysed and compared using the Kruskall-Wallis and the chi-square tests. RESULTS: Twenty-seven patients (25.7%) had clinical evidence of neurological involvement. They were 20 men and 7 women. The mean age at neurological onset was 34.26 +/- 8.79 years. Nineteen patients (70.3%) had meningoparenchymal "MP" central nervous system involvement (brainstem: 9, hemispheric involvement: 6, spinal cord: 4, psychiatric involvement: 2, isolated pyramidal signs: 1, aseptic meningitis: 1). Seven patients (25.9%) had cerebral large vessels involvement that is cerebral angio-Behçet "CAB" (intracranial hypertension: 5 cases due to cerebral venous thrombosis: 3 and pseudotumor cerebri: 2, cerebral haemorrhage: 1, cerebral arterial thrombosis: 1). One patient (3.7%) had both "MP" and "CAB" involvement. Headache was significantly more frequent in non-parenchymal patients. In group 1, complete recovery or improvement with mild neurological impairment was seen in 13 cases, improvement with severe disability in 3 cases, worsening in 1 case, the course was stationary in 1 case and 3 patients died (11.2%). Arterial aneurysms were significantly more frequent in "CAB" subgroups than in subgroup 2. CONCLUSION: Frequency of neurological involvements in BD was high in our study. Arterial aneurysms seem to be a risk factor to these complications. Cerebral angio-Behçet appears to be a protector factor against uveitis.
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INTRODUCTION: The occurrence of skin damage during systemic amyloidosis is common, but the appearance of bullous lesions is rare. Only twenty-seven cases have been reported in the literature. We report our observation of bullous amyloidosis during progression of renal amyloidosis. OBSERVATION: A 61 year-old man, presented with white, soft, palpebral edemas of the lower limbs, without scutulum involvement, associated with a large cubital nerve that had appeared in March 1997. Biological explorations revealed a nephrotic syndrome. Pathologic study of the renal biopsy concluded in amyloidosis. Treatment with colchinine stabilized the renal damage. One year later, a non-pruriginous, papular and bullous eruption occurred, localized essentially in the axillary and inguinal-crural folds of the forearms and legs. In the presence of an amyloidal deposit and intra-epidermal detachment, the cutaneous biopsy was evocative of bullous amyloidosis. The search for concomitant myeloma was negative. Treatment with colchinine was effective. The bullous lesions disappeared after 2 months, and 21 months later, renal damage was still stable. DISCUSSION: These particularities in evolution are exceptional and have never been described. A hypothetical modification in the physico-chemical properties of the amyloidal protein might explain the bullous eruption and stabilization of renal damage.