ABSTRACT
OBJECTIVES: To analyse the safety and efficacy of the off-label use of rituximab in patients with severe, refractory systemic autoimmune diseases. METHODS: In 2006, the Study Group on Autoimmune Diseases of the Spanish Society of Internal Medicine created the BIOGEAS project, a multicenter study devoted to collecting data on the use of biological agents in adult patients with systemic autoimmune diseases refractory to standard therapies (failure of at least two immunosuppressive agents). RESULTS: One hundred and ninety-six patients with systemic autoimmune diseases treated with rituximab have been included in the Registry (158 women and 38 men, mean age 43 years). Systemic autoimmune diseases included systemic lupus erythematosus (107 cases), inflammatory myopathies (20 cases), ANCA-related vasculitides (19 cases), Sjögren's syndrome (15 cases) and other diseases (35 cases). A therapeutic response was evaluable in 194 cases: 99 (51%) achieved a complete response, 51 (26%) a partial response and 44 (23%) were classified as non-responders. After a mean follow-up of 27.56+/-1.32 months, 44 (29%) out of the 150 responders patients relapsed. There were 40 adverse events reported in 33 (16%) of the 196 patients. The most frequent adverse events were infections, with 24 episodes being described in 19 patients. Thirteen (7%) patients died, mainly due to disease progression (7 cases) and infection (3 cases). CONCLUSIONS: Although not yet licensed for this use, rituximab is currently used to treat severe, refractory systemic autoimmune diseases, with the most favourable results being observed in Sjögren's syndrome, inflammatory myopathies, systemic lupus erythematosus and cryoglobulinemia.
Subject(s)
Antibodies, Monoclonal, Murine-Derived/therapeutic use , Autoimmune Diseases/drug therapy , Immunologic Factors/therapeutic use , Off-Label Use , Severity of Illness Index , Adolescent , Adult , Aged , Aged, 80 and over , Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/drug therapy , Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/ethnology , Antibodies, Monoclonal, Murine-Derived/adverse effects , Autoimmune Diseases/ethnology , Cryoglobulinemia/drug therapy , Cryoglobulinemia/ethnology , Female , Humans , Immunologic Factors/adverse effects , Lupus Erythematosus, Systemic/drug therapy , Lupus Erythematosus, Systemic/ethnology , Male , Middle Aged , Myositis/drug therapy , Myositis/ethnology , Retrospective Studies , Rituximab , Sjogren's Syndrome/drug therapy , Sjogren's Syndrome/ethnology , Spain , Treatment Outcome , Young AdultABSTRACT
We evaluated the influence of the hereditary make-up on the development of systemic lupus erythematosus (SLE) in two ethnic groups [Gypsy and white Caucasian Mediterranean (WCM) populations], living in the same geographic area. We compared 81 WCM and 25 Gypsy patients with SLE. The control group consisted of 185 healthy unrelated individuals, 105 WC and 80 Gypsies. In the Gypsy population, the onset of SLE occurred at earlier ages than in the other ethnic group (25.9 versus 32.0 years, P = 0.02), and showed lower SLEDAI peak values (4.9 versus 7.0, P = 0.016). The frequency of joint, kidney, gastrointestinal and eye involvement was significantly lower in Gypsy patients. In contrast, SLE-associated antiphospholipid syndrome, thrombosis and livedo reticularis were more frequent in Gypsies than in the majority ethnic group (WCM). In WCM patients, DRB1* 1303-DQB1*0301 haplotype was associated with SLE (P = 0.001, Pc = 0.038). We found SLE to be associated with DR5 (P = 0.006, Pc = 0.05) in the Gypsy population as well as a protective effect of DPB1*0401 when DR5 was not present (P = 0.008, Pc = 0.032). In conclusion, we found some clinical differences between WCM and Gypsy patients with SLE. Furthermore, HLA associations between HLA-DRB1-DQB1 and SLE were different for Gypsy people.
Subject(s)
HLA-DP Antigens/genetics , HLA-DQ Antigens/genetics , HLA-DR Antigens/genetics , Lupus Erythematosus, Systemic/ethnology , Roma/genetics , White People/genetics , Adult , Female , HLA-DP beta-Chains , HLA-DQ beta-Chains , HLA-DRB1 Chains , Humans , Lupus Erythematosus, Systemic/etiology , Male , Mediterranean Region/ethnology , Middle Aged , Severity of Illness Index , SpainABSTRACT
Propósito: La asociación entre las enfermedades autoinmunes y linfomas es por todos bien conocida aunque la aparición suele ser posterior, por ello es de suma importancia la sospecha de esta relación en todo paciente con estos antecedentes y clínica de un síndrome linfoproliferativo (SLP). Material y métodos: Se presenta el caso de un varón de 68 años diagnosticado de lupus eritematoso sistémico (LES) y desarrollo a los 18 meses de un linfoma no Hodgkin (LNH) de alto grado. Resultados y conclusiones: Se postula si la terapia inmunosupresora recibida por los pacientes con enfermedades autoinmunes puede influir en el desarrollo posterior de un SLP. Se discute si, ante cualquier síntoma sospechoso es necesario la realización precoz de todas las pruebas diagnósticas para descartar un linfoma (AU)
Subject(s)
Aged , Male , Humans , Lupus Erythematosus, Systemic/complications , Lymphoma, Non-Hodgkin/complications , Lymphoma, Non-Hodgkin/diagnosis , Severity of Illness Index , Fatal OutcomeSubject(s)
Anemia, Iron-Deficiency/diagnosis , Celiac Disease/diagnosis , Heart Block/diagnosis , Adult , Chronic Disease , Female , HumansSubject(s)
Behcet Syndrome/complications , Epididymitis/etiology , Orchitis/etiology , Adult , Appendicitis/etiology , Humans , Male , Oral Ulcer/etiology , Recurrence , Ulcer/etiology , Uveitis/etiologyABSTRACT
Jin Shin Do Bodymind Acupressure is the synthesis of Western psychology with Japanese acupressure technique, Chinese acupuncture theory, and Taoist philosophy and technique. Oriental health care philosophy provides a knowledge base for nurses who offer acupressure as an alternative health care choice. Acupressure provides a bodymind approach to pain management and a unique processing method in mental health counseling.
Subject(s)
Acupuncture Points , Complementary Therapies , Massage/methods , Mental Health , Counseling , Humans , Medicine, East Asian Traditional , Philosophy , PressureABSTRACT
Cyclic neutropenia is a rare hematological disorder consisting of recurrent episodes of aphthous stomatitis and skin infections caused by a periodic decrease in blood neutrophil counts. We present the case of such a patient successfully treated with steroids. Recurrent aphthous stomatitis with a periodicity of around 3 weeks should alert the dermatologist to the possibility of cyclic neutropenia.
