Therapeutic plasma exchange for the management of refractory systemic autoimmune diseases: report of 31 cases and review of the literature.

INTRODUCTION: Therapeutic plasma exchange (TPE) represents a treatment option in patients with systemic autoimmune diseases because most of their clinical manifestations are related to the presence of antibodies or immune complex deposition. OBJECTIVE: To describe the main demographic and clinical characteristics as well as the outcome of patients with systemic autoimmune diseases treated with TPE at a tertiary care center. METHODS: We included all patients with systemic autoimmune diseases in whom the indication for treatment with TPE was a flare of the disease between 1999 and 2010. The indications for treatment, complications and outcomes were obtained from review of medical records. RESULTS: A total of 31 patients (18 (58%) females and 13 (42%) males) were treated with a total of 196 TPE sessions with an average of 6.3 sessions per patient. Mean age at the time of TPE was 52.9 years (range, 26.0-82.0 years). Ten (32.3%) patients had ANCA-associated vasculitides, 6 (19.4%) mixed cryoglobulinemia secondary to hepatitis C virus (HCV) infection, 5 (16.1%) essential mixed cryoglobulinemia, 4 (12.9%) catastrophic antiphospholipid syndrome, 3 (9.7%) systemic lupus erythematosus, 2(6.5%) dermatomyositis and 1 (3.1%) polyarteritis nodosa. All patients except one were receiving corticosteroids at varying doses and all received a concomitant immunosuppressive drug. Ten (32.3%) and 9 (29.0%) patients received rituximab and intravenous immunoglobulins prior to TPE, respectively. Six patients experienced catheter-related infections, 5 urinary infections and 3 patients developed hospital acquired pneumonia. Eleven patients died in spite of the TPE. CONCLUSIONS: TPE is an effective therapeutic option for treating serious manifestations of systemic autoimmune diseases and a valid option for those patients with refractory disease to conventional treatments.

Epidemiology and management of refractory lupus nephritis.

Although the survival of patients with lupus nephritis (LN) has improved considerably in recent years, refractory LN appears in a substantial proportion of patients and, therefore, treatment of LN remains a real challenge today. We will use the term "refractory" LN, for those cases with none or partial response to first-line therapies. In this sense, numerous epidemiological factors, including racial, socioeconomic, histological and serological parameters, may influence treatment response and, therefore, may have an impact on the outcome of renal involvement. Initial conventional therapy will depend somewhat on these epidemiological factors. If this initial therapy fails, fortunately today we have alternative therapies that include the multitarget therapy and the use of biologics. Published evidence about these therapies is presented in this review. Important terms in the management of LN, such as the definition of complete response, partial response, sustained response and renal flare as well as the discrimination of different types of flare, are also discussed here according to the European consensus statement on the terminology used in the management of lupus glomerulonephritis.

Heart valve surgery in patients with the antiphospholipid syndrome: analysis of a series of nine cases.

OBJECTIVE: Antiphospholipid syndrome (APS) is a rare coagulation disorder associated with recurrent arterial and venous thrombotic events. Heart valve abnormalities are commonly found in patients with APS. METHODS: From March 1998 to March 2007, nine patients with APS underwent heart valve surgery using cardiopulmonary bypass. We retrospectively reviewed their clinical data, operative and postoperative courses and the long-term results. RESULTS: The mean age was 43.6+/-10.4 years, six were female and three male. Four patients underwent mitral valve replacement, three went through aortic valve replacement, one underwent combined mitral-aortic valve replacement and another aortic valve plasty. The syndrome was primary in seven patients and associated with systemic lupus erythematosus (SLE) in two. Follow-up was 8 days to 8 years (median 66 months). Two patients died in the early postoperative period: both due to an acute cerebrovascular accident. Four patients presented an uneventful late postoperative course. One patient experienced an ischaemic stroke 5 years after mitral valve replacement (MVR) and developed refractory congestive heart failure requiring heart transplantation three years postoperatively. CONCLUSIONS: Heart valve surgery in patients with antiphospholipid syndrome may carry considerable early and late mortality and morbidity. Thrombo-embolic complications are the most common complications. Mechanical prostheses have been used at our Institution in the previous years; however, today, after reviewing our historical results, we reconsider our general strategy and believe that tissue heart valve prostheses are the possible ideal substitutes, minimising the risks of morbidity and mortality due to the hypercoagulable state of APS.

Catastrophic antiphospholipid syndrome (CAPS): descriptive analysis of a series of 280 patients from the "CAPS Registry".

OBJECTIVE: To describe the clinical and laboratory features, as well as the precipitating factors, treatment and outcome of patients with catastrophic antiphospholipid syndrome (APS). METHODS: We analyzed the 280 patients included until September 2008 in the website based international registry of patients with catastrophic APS ("CAPS Registry") (http://www.med.ub.es/MIMMUN/FORUM/CAPS.HTM). RESULTS: The entire series includes 201 (72%) female and 79 (28%) male patients with a mean age of 37 +/- 14 years (range, 11-60 years). A total of 129 (46%) patients suffered from primary APS, 112 (40%) from systemic lupus erythematosus, 14 (5%) from lupus-like disease, and 25 (9%) from other autoimmune diseases. The catastrophic episode was the first manifestation of the APS in 129 (46%) patients. A precipitating factor was reported in 53% of the patients. The first clinical manifestation at the time of the catastrophic episode was a pulmonary complication in 24% of the cases, a neurologic feature in 18% and a renal feature in 18%. During the catastrophic episode, intraabdominal involvement was identified in the majority of patients, mainly consisting of renal (71%), hepatic (33%), gastrointestinal (25%), splenic (19%), adrenal (13%), and pancreatic (8%) manifestations. 123 (44%) patients died at the time of the catastrophic APS event but the higher recovery rate was achieved by the combination of anticoagulants plus corticosteroids plus plasma exchange (PE) and/or intravenous immunoglobulins (IVIG) (69% versus 54%). CONCLUSIONS: The catastrophic APS is an uncommon but potentially life-threatening condition that needs high clinical awareness. The therapeutical connotation is that this may be corrected with the combination of anticoagulation plus steroids plus attempts at achieving a prompt reduction of antiphospholipid antibody titer (i.e. PE and/or IVIG).