Florian Pasquet

Uveitis and Common Variable Immunodeficiency: Data from the DEF-I Study And Literature Review

Purpose: To examine the relation between uveitis and common variable immunodeficiency (CVID). Methods: Retrospective analysis of patients included in the French DEFI cohort of adults with CVID and of patients identified by reviewing the literature. Results: Four patients were identified in the DEFI study (frequency of uveitis: 1.6%). The course of uveitis was not changed in the patients who started intravenous immunoglobulins replacement therapy after CVID diagnosis. Ten cases of CVID-associated uveitis were listed in the literature. Overall, among the 14 patients, uveitis was always chronic, usually bilateral (n = 11) and granulomatous (n = 9). Seven patients presented with a “sarcoid-likeâ syndrome. Nine patients had granulomatous uveitis suggestive of ocular sarcoidosis. Five patients were treated with local corticosteroids, and 9 required systemic treatment (corticosteroids alone n = 5 and/or immunosuppressive agents n = 4). Conclusions: CVID can be associated with uveitis. Since uveitis can reveal the immunodeficiency, CVID diagnosis should be considered at the time of uveitis diagnosis.

Coexisting relapsing polychondritis and sarcoidosis: an unusual association.

Relapsing polychondritis is an episodic and progressive systemic inflammatory disease characterized by auricular chondritis, polyarthritis, nasal and respiratory tract chondritis. About 30% of the patients have additional autoimmune and or hematological diseases, most frequently systemic vasculitis, rheumatoid arthritis, myelodysplastic syndromes or systemic lupus erythematosus. So far, only one case of coexisting relapsing polychondritis and sarcoidosis in a patient with AIDS has been reported. We describe here a case of sarcoidosis and relapsing polychondritis in an immunocompetent patient. Physicians should be aware of this possible association.

Granulomatoses rénales : étude rétrospective de 44 observations

PURPOSEnGranulomatous interstitial nephritis (GIN) are identified in 0.5 to 1,3% of all renal biopsies. Renal outcome and treatment modalities are not clearly established in the literature.nnnMETHODSnWe retrospectively analyzed a case series of 44 GIN identified among all renal biopsies performed between 1984 and 2005 in the Rhône-Alpes area.nnnRESULTSnThe study population included 25 men and 19 women with a mean age of 56 years, and mean diagnostic delay was 11 months. Renal function was severely impaired (mean creatinine clearance 24mL/min). Proteinuria was observed in 77% (mean value 0,9xa0g/24h) of the patients and associated with microscopic hematuria and leukocyturia in 30% and 25%, respectively. The most common diagnosis was sarcoidosis (25%, nxa0=xa011), followed by drug-induced GIN (9%, nxa0=xa04), tuberculosis (6,8%, n=3), hemopathy-related paraneoplastic GIN (6,8%, nxa0=xa03), HIV infection (nxa0=xa01) and chronic renal allograft rejection (nxa0=xa01). In other patients, no aetiology was found (48%, nxa0=xa021). Severity of renal failure justified hemodialysis in 34% (nxa0=xa015) of the patients. Three patients underwent renal transplantation. Nonetheless, renal outcome was generally favorable: renal function improved in 41% (nxa0=xa018) and stabilized in 34% (nxa0=xa015) of patients.nnnCONCLUSIONSnSarcoidosis, drug-induced and infections represent the main causes of GIN. Histologic features are not specific enough to determine the aetiology. Corticosteroids is the gold standard in sarcoidosis, drug-induced, and idiopathic GIN. Treatment is etiologic in the other cases.

Axitinib-induced acute pancreatitis: a case report.

Axitinib is an oral second-generation selective inhibitor of vascular endothelial growth factor receptors recently approved for the treatment of advanced renal cell carcinoma. Numerous cases of acute pancreatitis have been reported after treatment with nonselective tyrosine kinase inhibitors such as sorafenib and sunitinib. We present the first report of a patient under axitinib treatment presenting with acute pancreatitis for which no other etiology has been found. The patient was a 29-year-old woman treated for renal cell carcinoma. The patient had no history of chronic illness, gallstone-related disease, or alcohol consumption. She had been previously treated with sunitinib and everolimus. Four months after the onset of axitinib treatment she was hospitalized for acute pancreatitis. Symptoms and blood lipase levels normalized within a few days after axitinib was withheld. We believe that acute pancreatitis should be recognized as a potential axitinib-related adverse event.

Granulomatosis and Cancer

Sarcoidosis is a granulomatous disease of unknown cause that can virtually affect any organ system. The commonly affected organ areas are thoracic lymph nodes and lungs (90 %). The granulomas appears to be due to an aberant immune response to a persistent antigen in a susceptible host; the antigen, however, is yet to be identified(Shigemitsu, 2008). The diagnosis typically rests on the demonstration of characteristic granulomas in biopsy specimens of one or more organs and exclusion of other causes of chronic granulomatous inflammation(Boffetta et al., 2009; Pavic et al., 2008b). Sarcoidosis affects more frequently young adults. Genetic studies have shown that some major histocompatibility complex alleles and tumor necrosis factor (TNF) polymorphisms are associated with an increased risk of sarcoidosis. The disease is usually characterized by an increased macrophage and CD4 Tcell activation, whereas sarcoidosis patients also show suppressed response to antigen challenges. The coexistent of hyperand hypoactivity indicates a state of anergy in the immune system(Ji et al., 2009). Relationships betwen granulomatosis and cancers have been suspected for a long time(Askling et al., 1999; Brincker and Wilbek, 1974; Pavic and Rousset, 2008; Romer et al., 1998). Nevertheless, few evidence has been reported until recently. Aside from granulomatosis due to infectious disease (eg: opportunist infections), granulomas can be observed in cancer patients, mainly in two situations. Patients may rarely present with typical sarcoidosis occuring before, during or after the diagnosis of cancer. Secondly granulomas may be found as a sarcoid reaction in the vicinity of the tumour itself ore more frequently in regional lymph nodes(Pavic et al., 2008a). Sarcoidosis has although been reported to occur with some chemotherapeutic agents or immunotherapy such as interferon. The presence of granulomas within the tumor tissue or in regional ganglions is a frequent situation and corresponds to a defense reaction against the tumor-associated antigen (sarcoid like reaction)(Kennedy et al., 2008; Steinfort and Irving, 2009). The precise mechanism of the sarcoid-cancer syndrome is not yet clear, though there are several descriptions in the literature as to the temporal relationship of granulomas developing in cancer and vice versa. The etiology of sarcoidosis and sarcoid reactions in malignancy remains uncertain but some speculations have been made(Trikudanathan et al.): 1/ induction of robust effector T cell response to a tumor antigen or other products of cancer cells either spontaneously or with treatment that result in T helper 1 response along with secretion of TH1 cytokines; 2/ increased vulnerability to a potential infective agent due to immune system imbalance that occurs with cancer; 3/ radiation therapy and antineoplastic agents might enhance granulomatous reactions in tumors; 4/granulomatous reaction could

Accident hémolytique retardé post-transfusionnel par allo-immunisation anti-JK1

INTRODUCTIONnThe sensitivity of the detection of irregular antibodies (DIA) is one of the fundamental basis of transfusion safety. The production of alloantibodies is the first cause of adverse events following transfusion.nnnCASE REPORTnWe report a 77-year-old woman who was transfused and presented with a delayed haemolytic anemia due to anti-JK1 alloimmunization. This event highlights the limits of DIA performed before a transfusion, the hazard of this specific type of antibody and the difficulties of the diagnosis of haemolytic anaemia. The preventive measures necessary to avoid this undesirable effect are reminded.nnnCONCLUSIONnDespite the sensitive routine test method, the anti-JK1 antibodies could be missed. We should keep in mind the possibility of an anaemia due to alloantibodies we confronted to an unexplained haemolytic episode.

Communication brèveAccident hémolytique retardé post-transfusionnel par allo-immunisation anti-JK1Delayed haemolytic transfusion reaction due to anti-JK1 antibody

INTRODUCTIONnThe sensitivity of the detection of irregular antibodies (DIA) is one of the fundamental basis of transfusion safety. The production of alloantibodies is the first cause of adverse events following transfusion.nnnCASE REPORTnWe report a 77-year-old woman who was transfused and presented with a delayed haemolytic anemia due to anti-JK1 alloimmunization. This event highlights the limits of DIA performed before a transfusion, the hazard of this specific type of antibody and the difficulties of the diagnosis of haemolytic anaemia. The preventive measures necessary to avoid this undesirable effect are reminded.nnnCONCLUSIONnDespite the sensitive routine test method, the anti-JK1 antibodies could be missed. We should keep in mind the possibility of an anaemia due to alloantibodies we confronted to an unexplained haemolytic episode.