ABSTRACT
Autoimmune hemolytic anemias (AIHAs) are rare and heterogeneous disorders characterized by the destruction of red blood cells by warm or cold antibodies. Hemolytic anemia associated with warm antibodies is the most common, whereas cold antibodies are rare and infrequent in cases published in the scientific literature. Herein, we present the case of a young patient with systemic lupus erythematosus (SLE) and autoimmune hemolytic anemia caused by cold antibodies. Initially, infectious etiology and hematological malignancy were considered, which were ruled out. She required management in the intensive care unit due to severe hematological involvement and responded well to immunomodulatory therapy. This case illustrates the importance of a strong clinical suspicion of AIHA due to cold agglutinins associated with SLE when faced with similar clinical symptoms in order to achieve a timely diagnosis and provide optimal therapy.
ABSTRACT
BACKGROUND/OBJECTIVE: Data on IgG4-related disease (IgG4-RD) come almost exclusively from cohorts from Asia, Europe, and North America. We conducted this study to describe the clinical presentation, phenotype distribution, and association with sex, ethnicity, and serological markers in a large cohort of Latin American patients with IgG4-RD. METHODS: We performed a multicenter medical records review study including 184 Latin American IgG4-RD patients. We assigned patients to clinical phenotypes: group 1 (pancreato-hepato-biliary), group 2 (retroperitoneal/aortic), group 3 (head and neck-limited), group 4 (Mikulicz/systemic), and group 5 (undefined). We focused the analysis on how sex, ethnicity, and clinical phenotype may influence the clinical and serological presentation. RESULTS: The mean age was 50.8 ± 15 years. Men and women were equally affected (52.2% vs 48.8%). Fifty-four patients (29.3%) were assigned to group 1, 21 (11.4%) to group 2, 57 (30.9%) to group 3, 32 (17.4%) to group 4, and 20 (10.8%) to group 5. Male sex was associated with biliary tract (odds ratio [OR], 3.4; 95% confidence interval [CI], 1.36-8.26), kidney (OR, 3.4; 95% CI, 1.28-9.25), and retroperitoneal involvement (OR, 5.3; 95% CI, 1.45-20). Amerindian patients presented more frequently with atopy history and gallbladder involvement. Group 3 had a female predominance. CONCLUSIONS: Latin American patients with IgG4-RD were younger, and men and women were equally affected compared with White and Asian cohorts. They belonged more commonly to group 1 and group 3. Retroperitoneal and aortic involvement was infrequent. Clinical and serological features differed according to sex, ethnicity, and clinical phenotype.
Subject(s)
Immunoglobulin G4-Related Disease , Adult , Aged , Ethnicity , Female , Humans , Immunoglobulin G , Latin America , Male , Middle Aged , PhenotypeABSTRACT
RESUMEN La enfermedad de Paget ósea es una enfermedad metabólica del hueso de etiología no esclarecida, que se caracteriza por una fase de resorción aumentada seguida por una fase de osteoformación aberrante. Es frecuente en Europa, Norteamérica, Nueva Zelanda y Australia, pero infrecuente en Asia, Medio Oriente y África. En población colombiana hay reportes de casos. Generalmente cursa asintomática y se diagnostica incidentalmente por hallazgos radiográficos o fosfatasa alcalina elevada. El uso de bifosfonatos favorece el control del recambio óseo y permite prevenir complicaciones como las fracturas. Se presenta una serie de casos en Colombia y una revisión de la literatura.
A B S T R A C T Paget's disease of the bone is a metabolic bone disease of unknown origin, and is characterised by an increased phase of resorption, followed by an aberrant osteoformation phase. It is common in Europe, North America, New Zealand, and Australia, but infrequent in Asia, the Middle East, Africa, and in the Colombian population there are case reports. It is usually asymptomatic and is diagnosed incidentally by radiographic findings or an elevated alkaline phosphatase. The use of bisphosphonates favours the control of bone turnover and prevents complications such as fractures. A series of cases in Colombia is presented, along with a review of the literature.
Subject(s)
Humans , Middle Aged , Aged , Osteitis Deformans , Fractures, Bone , Population , Bone and Bones , Bone Diseases, Metabolic , DiphosphonatesABSTRACT
RESUMEN La arteritis de Takayasu es una de las vasculitis de grandes vasos que afecta a la aorta y a sus ramas principales, llevando a estenosis arterial y, en ocasiones, a trombosis. También se observa la formación de aneurismas en la circulación general. No tiene un tratamiento específico. La terapia de elección son los corticoides, seguidos de otros inmunosupresores en casos de no lograrse un control adecuado del proceso inflamatorio. Se reporta el caso de un paciente con arteritis de Takayasu, con formación inusual de aneurisma gigante de la arteria pulmonar.
ABSTRACT Takayasu arteritis is a large vessel vasculitis that affects the aorta and its main branches, leadingto arterial stenosis, and occasionally, thrombosis. There is also aneurysm formation in the general circulation. There is no specific treatment. The therapy of choice is corti-costeroids, followed by other immunosuppressants in cases where adequate control of the inflammatory process is not achieved. A case is presented of a patient with Takayasu arteritis and the unusual presence of giant aneurysm of pulmonary artery.
