1/8. Noncaseating granulomatous disease in common variable immunodeficiency.patients with common variable immunodeficiency (CVID) are occasionally recognized to have a concurrent noncaseating granulomatous disease. The granulomatous disease (GD) associated with CVID shares many clinical properties typical of sarcoidosis. Some investigators speculate that the GD-CVID is actually sarcoidosis that is expressed atypically because of the patient's immunodeficiency. Clinical differences, however, have led other investigators to speculate that the GD-CVID is a distinct "sarcoid-like" granulomatous process.- - - - - - - - - - ranking = 1keywords = granulomatous disease (Clic here for more details about this article) |
2/8. Granulomatous renal disease in a patient with common variable immunodeficiency.common variable immunodeficiency (CVID), the most common cause of primary hypogammaglobulinemia, is characterized by a decreased serum immunoglobulin level, recurrent infections, and the occurrence of various autoimmune diseases. Granulomatous disease has been reported previously in several patients with CVID, with granuloma occurring in the lymph nodes, spleen, liver, central nervous system, and bone marrow. We report the first published case of renal granulomatous disease in a CVID patient presenting with subacute renal failure. Renal function partially recovered after corticosteroid treatment and intravenous immunoglobulin infusions. The pathogenesis of granulomatous disease in CVID is unclear but may involve monocyte and T-cell abnormalities.- - - - - - - - - - ranking = 0.33333333333333keywords = granulomatous disease (Clic here for more details about this article) |
3/8. A unique renal lesion in common variable immunodeficiency.This article reports the case of a 33-year-old woman with common variable immunodeficiency (CVI) who developed renal failure 17 years after diagnosis and initiation of treatment with monthly IVIG. A renal biopsy revealed mesangial and paramesangial immune complex deposition and interstitial granulomatous infiltration. Renal function improved with oral corticosteroids, but did not return to normal. Decreasing the dose of IVIG had no effect on renal function. Immune dysfunction can be associated with both granulomatous disease and immune complex glomerulonephritis, or the latter may be related to chronic infection or immunoglobulin use. This is the first report of concomitant glomerular-tubulointerstitial lesions in this immunodeficiency syndrome. Renal function should be closely followed in patients with CVI.- - - - - - - - - - ranking = 0.16666666666667keywords = granulomatous disease (Clic here for more details about this article) |
4/8. Gammadelta T lymphocytosis associated with granulomatous disease in a patient with common variable immunodeficiency.common variable immunodeficiency (CVID) is a heterogeneous group of immunodeficiency syndromes that involves defective production of specific antibodies and decreased serum concentrations of > or =1 immunoglobulin isotype. We describe a patient with an atypical case of CVID who had extensive granulomatous lesions that were partially attributable to mycobacterial infection. In the peripheral blood, there was a massive increase in the number of double-negative CD3 T cells that expressed the gammadelta T cell receptor.- - - - - - - - - - ranking = 0.66666666666667keywords = granulomatous disease (Clic here for more details about this article) |
5/8. lymphoma complicating common variable immunodeficiency with granulomatous disease: report of two cases.common variable immunodeficiency (CVID) is a primary defect that is characterized by impaired antibody production. CVID patients may develop recurrent infections, autoimmune disorders and/or systemic granulomatosis. It is well documented that CVID patients are at risk to develop malignant lymphomas. However, to the best of our knowledge, lymphoma complicating the course of CVID associated with systemic granulomatosis has never been reported. We describe two CVID patients with systemic granulomatosis who developed B-cell lymphomas, one related to Epstein Barr virus infection, 5 and 12 yr after CVID had been diagnosed.- - - - - - - - - - ranking = 0.66666666666667keywords = granulomatous disease (Clic here for more details about this article) |
6/8. Development of granulomatous common variable immunodeficiency subsequent to infection with toxoplasma gondii.common variable immunodeficiency (CVID) is a heterogeneous immunodeficiency that is accompanied by granulomatous lesions in 5-10% of cases. Why some patients develop granulomatous disease remains unclear. Here we describe a 12-year-old previously healthy girl who presented with pancytopenia and granulomatous lymphoproliferation subsequent to infection with toxoplasma gondii. Loosely arranged non-fibrosing granulomas were observed in the liver, lymph nodes and lung, but no toxoplasma tachyzoites could be demonstrated and polymerase chain reaction (PCR) and culture were negative for toxoplasma and a wide range of other pathogens. While the patient had a normal peripheral B cell status at presentation, the development of CVID could be observed during the following months, leading to a loss of memory B cells. This was accompanied by an increasingly activated CD4( ) T cell compartment and high serum levels of angiotensin-converting enzyme (ACE), tumour necrosis factor (TNF) and sCD25. Steroid therapy reduced pancytopenia, granulomatous lymphoproliferation and cytokine elevations, but did not improve the B cell status. This is the first report of an association of toxoplasma infection with granulomatous CVID and provides one of the rare examples where the onset of CVID could be documented subsequent to an infectious disease.- - - - - - - - - - ranking = 0.16666666666667keywords = granulomatous disease (Clic here for more details about this article) |
7/8. Infliximab treatment for severe granulomatous disease in common variable immunodeficiency: a case report and review of the literature.BACKGROUND: Granulomatous disease resembling sarcoidosis is a well-described condition associated with common variable immunodeficiency (CVID). Its treatment remains problematic, and new therapeutic options are needed. OBJECTIVES: To report the efficacy of treatment with infliximab, a chimeric anti-tumor necrosis factor alpha monoclonal antibody, in a patient with granulomatous CVID and to review the literature on the treatment of patients with granulomatous CVID. methods: A 22-year-old white man with CVID developed acute multiorgan failure, with granulomatous inflammation on lung and liver biopsy specimens. He was initially treated with antibiotics, intravenous immunoglobulin, and corticosteroids for 5 weeks without improvement. High-dose infliximab was then infused weekly for 6 weeks and then monthly for 9 months. The response to infliximab was determined by changes on clinical examination, imaging studies, and histologic studies. RESULTS: The patient's condition dramatically improved after 1 dose of infliximab infusion, with decreasing hepatosplenomegaly, ventilatory support requirements, and pulmonary infiltrates. Ventilatory support was successfully discontinued within 3 weeks. The corticosteroid dose was tapered without reactivation of the disease. After 9 months of therapy, follow-up imaging studies showed resolution of pulmonary infiltrates, no hepatosplenomegaly, and no portal hypertension, and a percutaneous liver biopsy revealed no granulomas; then, infliximab use was discontinued. The patient remains free of granulomatous disease after 18 months of follow-up. CONCLUSIONS: To our knowledge, this is the first report of severe visceral granulomatous CVID successfully treated with infliximab. Infliximab may be an effective therapy for granulomas in CVID. Further studies of infliximab and other tumor necrosis factor a antagonist therapies in granulomatous CVID are warranted.- - - - - - - - - - ranking = 0.66666666666667keywords = granulomatous disease (Clic here for more details about this article) |
8/8. Asymptomatic choroidal granulomas in common variable immunodeficiency.common variable immunodeficiency disease represents the most common cause of significant antibody deficiency in adults. It is a heterogeneous group of disorders that can be sporadic or familial with various modes of inheritance with defects in B-cell function, regulatory T-cells and macrophage function all being reported. Herein a case of asymptomatic bilateral choroidal granulomatous disease in a 27-year-old man with common variable immunodeficiency disease is described.- - - - - - - - - - ranking = 0.16666666666667keywords = granulomatous disease (Clic here for more details about this article) |