Mohamed Habib Houman

Association of right heart thrombosis, endomyocardial fibrosis, and pulmonary artery aneurysm in Behçet’s disease

A 29-year-old man with a 5-year history of Behçets disease was admitted for fever, dyspnae, chest pain, and hemoptysis. A diagnosis of right ventricle and atrial thrombosis associated with a pulmonary artery aneurysm was made. The patient was treated with anticoagulants and prednisone. Since hemoptysis persisted, surgical excision of the intracardiac thrombosis was performed and histological findings were consistent with organizing thrombus and endomyocardial fibrosis. Transesophageal echocardiography 6 months later showed recurrence of the right ventricle thrombosis. A course of 6-monthly boluses of intravenous cyclophosphamide was begun. Currently, at 2 years of follow-up, the patient is asymptomatic.

TULUP (TUnisian LUPus): a multicentric study of systemic lupus erythematosus in Tunisia.

Clinical features of systemic lupus erythematosus (SLE) have been described from different geographical regions around the world. However, data from North African countries, including Tunisia, are scarce.

Contribution of Hla-B51 in the susceptibility and specific clinical features of Behcet's disease in Tunisian patients

BACKGROUND Many researchers have tried to investigate the association of HLA-B51 with the severity and the clinical features of BD with conflicting results. METHODS We aimed at investigating the association of HLA-B51 with demographical and clinical manifestations as well as the severity of BD, by studying 178 native Tunisian BD patients, fulfilling the International Study group criteria for the BD classification recruited from the Department of Internal Medicine, Rabta Hospital in Tunis and compared with 125 native Tunisian healthy age and sex matching volunteers. RESULTS According to our findings, the frequency of HLAB 51 was significantly higher in BD patients than in controls (p<0.001). Positive pathergy test (PPT) (p = 0.01) and retinal vasculitis (p = 0.045), were significantly more frequent in HLA B51(+) patients, while the frequency of arterial aneurysms (p = 0.009) and neurological involvement, especially the parenchymal involvement (p<0.001), were significantly and clearly higher in HLA B51(-) patients. The patients without HLA B51 had a significantly less severe disease (p = 0.001). Discussion/conclusion We conclude that HLA B51 is a predisposing marker for BD in our population as in most ethnic groups. It seems to be associated with a subgroup of BD patients characterized by a higher frequency of ocular involvement and PPT, but a lower frequency of arterial aneurysm and neurological involvement, and a less severe disease course.

Resistance to Exogenous TGF-β Effects in Patients with Systemic Lupus Erythematosus

BackgroundThe mechanisms underlying the loss of self-tolerance in systemic lupus erythematosus (SLE) are incompletely deciphered. TGF-β plays a key role in self-tolerance demonstrated by the onset of a fatal autoimmune syndrome associated with lupus autoantibodies in mice lacking a functional TGF-β receptor. The present work aims to define whether resistance to TGF-β might contribute to the pathogenesis of SLE.MethodsTwenty-two patients with active SLE, 16 with other connective tissue diseases, and 10 healthy controls were prospectively included in this study. The effects of exogenous TGF-β1 on IL-2-dependent T-cell proliferation, IFN-γ secretion, and target gene transcription were analyzed on peripheral blood mononuclear cells.ResultsOur results showed that 75% of patients with SLE or other connective tissue diseases were totally or partially resistant to the effects of TGF-β1. The responses to the anti-proliferative and transcriptional effects of TGF-β were, however, discordant in a high proportion of our patients. Hence, we distinguish three distinct profiles of resistance to TGF-β1 and suggest that patients may exhibit different defects affecting distinct points of TGF-β1 signaling pathways.ConclusionOur data demonstrate the presence of an impaired response of peripheral cells to TGF-β1 in patients with active SLE that may participate to the pathogenesis of the disease. Further studies will be necessary to delineate the mechanisms underlying the lymphocyte resistance to TGF-β1 in SLE.

Pleural myelomatous involvement in multiple myeloma: five cases.

Pleural myelomatous involvement in multiple myeloma (MM) is rare, occurring in less than 1% of cases. We retrospectively studied five cases of patients with MM who developed myelomatous pleural effusions. Three men and 2 women with a mean age of 61 years presented with myelomatous pleural effusion. The pleural fluid electrophoresis revealed a peak of IgG in three cases, of IgA in one case, and of lambda light chains in one case, which were identical to that in the sera of the patients. Detection of typical plasma cells in pleural fluid cytology was contributive, and histologic confirmation by pleural biopsy was positive in four cases. Treatment consisted of chemotherapy. The clinical outcome was initially good, but relapses occurred in all cases early and were complicated by fatal infections. Myelomatous pleural effusion is a rare affection. It is usually a late complication associated with poor prognosis.

Sténose urétérale au cours de la granulomatose de Wegener: Une observation

INTRODUCTION Ureteral stenoses in Wegeners granulomatosis are rare. They usually involve the pelvic portion of the ureter and are caused by vasculitic lesions or granulomatous inflammation. CASE A 38-year-old woman with Wegeners granulomatosis was treated with corticosteroids and monthly intravenous cyclophosphamide pulses. After 4 months, urinary retention developed, accompanied by lumbar pain, associated with protenuria and hematuria, and related to bilateral ureteral stenoses. Treatment by endoscopic dilatation and double J stents led to with clinical and radiological improvement, while the medical treatment continued. CONCLUSION Hematuria in patients with Wegeners granulomatosis suggests renal involvement, but ureteral stenoses must also be considered when these patients present hematuria or urinary tract infections. Surgery should be reserved for those patients in whom medical treatment is not rapidly effective.Recu le 8 aout 2005 Accepte le 20 juin 2006 ■ Summary Ureteral stenosis in Wegeners granulomatosis: Case report Introduction > Ureteral stenoses in Wegeners granulomatosis are rare. They usually involve the pelvic portion of the ureter and are caused by vasculitic lesions or granulomatous inflammation. Case > A 38-year-old woman with Wegeners granulomatosis was treated with corticosteroids and monthly intravenous cyclophospha- mide pulses. After 4 months, urinary retention developed, accompa- nied by lumbar pain, associated with protenuria and hematuria, and related to bilateral ureteral stenoses. Treatment by endoscopic dila- tation and double J stents led to with clinical and radiological impro- vement, while the medical treatment continued. Conclusion > Hematuria in patients with Wegeners granulomatosis suggests renal involvement, but ureteral stenoses must also be consi- dered when these patients present hematuria or urinary tract infec- tions. Surgery should be reserved for those patients in whom medi- cal treatment is not rapidly effective.

Panniculite au cours d’un traitement d’une dermatomyosite par du méthotrexate

Panniculitis is a rare cutaneous manifestation of dermatomyositis (DM). The appearance of panniculitis during treatment with methotrexate (MTX) is exceptional and has only been described in 3 cases. We report a case of a 50-year-old woman suffering from DM since 1997 who was treated with corticosteroids showing favorable clinical and biological evolution. When a relapse occurred 2 years later, she was treated with higher-dose of corticosteroids in combination with a 7,5 mg weekly dose of methotrexate. The evolution was rapidly favorable. Eighteen months later, the patient had multiple subcutaneous nodules on limbs and buttocks. Anatomopathological examination showed panniculitis. There was no evidence supporting progression in DM. Prednisone dose was increased to 0.5 mg/kg/day, always in combination with MTX, without any clear signs of improvement. MTX treatment was stopped and the cutaneous lesions completely disappeared in 2 months without any relapse. This objective response lasted for 42 months. Our observation is particular given the occurrence of panniculitis in a patient undergoing treatment for dermatomyositis with methotrexate and illustrates the difficulties in the diagnosis. This entity must be known despite its exceptional nature since cutting off MTX treatment generally induces the disappearance of subcutaneous nodules.

Central Retinal Vein Occlusion Revealing Coelic Disease

Introduction Thrombosis has been widely reported in coeliac disease (CD) but central retinal vein occlusion (CRVO) is rarely described. Case presentation A 27-year-old woman presented with acute visual loss and was diagnosed with CRVO. Her protein S and protein C levels were low and CD was diagnosed on the basis of endoscopic, immunological and histological results. A gluten-free diet resulted in favourable evolution. Conclusion CD should be considered in young patients with thrombosis, especially if in an unusual location. Treatment is based on a gluten-free diet. LEARNING POINTS Coeliac disease (CD) should be considered in young patients with central retinal vein occlusion (CRVO). Several mechanisms can cause thrombosis in CD. CRVO in CD is often reversible with a gluten-free diet.

Multiple Autoimmune Syndrome: Celiac Disease, Hashimoto's Thyroiditis, and Systemic Lupus Erythematosus

Multiple autoimmune syndrome is the association of three or more autoimmune diseases in the same patient. We report the case of a 24-year-old woman with a medical past history of celiac disease since the age of 3. She complained of fatigue, malar rash, and paresthesia of the lower limbs. Physical examination objectified fever at 38°C and butterfly rash. Cell blood count showed leucopenia and lymphopenia. Thyroid tests concluded to primary hypothyroidism. Immunological investigations revealed positive antinuclear antibodies (1/3200), anti-DNA, and thyroperoxydase antibodies. The HLA haplotype typing was A1 A26 B50 B16 DR3 DR52 DQ2. The diagnosis of multiple autoimmune syndrome associating systemic lupus erythematosus, celiac diasese, and Hashimoto’s thyroiditis was retained. The patient was treated with hydroxychloroquine 400 mg/day and L-thyroxine 100 µg/day. The global outcome was favorable and the patient was free of symptoms. The decline is 10 years. The current case is unique in this aspect that the combination of these three autoimmune disorders has rarely been reported in the past.

Primary Sjögren’s syndrome complicated by anti-neutrophil cytoplasmic antibody-mediated crescentic glomerulonephritis

Ocular and oral dryness are the hallmark of Sjögrens syndrome (SS). However, SS can be associated with a variety of complications, affecting organs such as the liver, kidneys, lungs, muscle, and nervous system. Renal involvement has been usually in the form of tubulointerstitial nephritis. However, glomerulonephritis is rare in primary SS. We report three clinical cases of SS with anti-neutrophil cytoplasmic antibody-mediated crescentic glomerulo-nephritis treated with prednisone and cyclophosphamide, with favorable outcome.