mycophenolic-acid and Multiple-Organ-Failure

Paraneoplastic pemphigus (PNP) or paraneoplastic autoimmune multiorgan syndrome (PAMS) is a life-threatening autoimmune blistering disease commonly associated with lymphoproliferative neoplasms. This article focuses on current management strategies in PNP/PAMS, and reported instances of their treatment successes and failures. Due to the rarity of the condition and the high rates of treatment failure, no randomized control trials exist to guide the evidence-based treatment of this condition; all evidence to date on the efficacy of therapeutic modalities has been gained from individual case reports, small case series, and expert recommendations.

Topics: Adrenal Cortex Hormones; Alemtuzumab; Antibodies, Monoclonal, Humanized; Antibodies, Monoclonal, Murine-Derived; Antibodies, Neoplasm; Autoimmune Diseases; Azathioprine; Cyclophosphamide; Cyclosporine; Dermatologic Agents; Female; Humans; Immunoglobulins, Intravenous; Immunosuppressive Agents; Male; Multiple Organ Failure; Mycophenolic Acid; Paraneoplastic Syndromes; Pemphigus; Plasmapheresis; Rituximab; Tacrolimus

Macrophage activation syndrome (MAS) is a severe and under-recognized complication of rheumatologic diseases. We describe a patient who presented with rapidly progressive, refractory MAS found to have anti-MDA5 antibody Juvenile Dermatomyositis (JDM) as her underlying rheumatologic diagnosis.. We describe a 14-year-old female who at the time of admission had a history of daily fevers for 6 weeks and an unintentional sixteen-pound weight loss. Review of systems was significant for cough, shortness of breath, chest pain, headaches, sore throat, muscle aches, rash, nausea, and loss of appetite. An extensive initial workup revealed findings consistent with an autoimmune process. While awaiting results of her workup she had clinical decompensation with multi-organ system involvement including pancytopenias, interstitial lung disease, hepatitis, cardiac involvement, gastrointestinal distension and pain, feeding intolerance, extensive mucocutaneous candidiasis, and neuropsychiatric decline. Due to her decompensation, significant interstitial lung disease, and likely underlying rheumatologic condition she was started on high dose pulse steroids and mycophenolate. An MRI was performed due to her transaminitis and shoulder pain revealing significant myositis. Intravenous immunoglobulin was then initiated. The myositis antibody panel sent early in her workup was significant for anti-MDA5 and anti-SSA-52 antibodies. Despite high dose pulse steroids, mycophenolate, and IVIG, her disease progressed requiring escalating therapies. Ultimately, she responded with resolution of her MAS as well as significant and steady improvement in her feeding intolerance, interstitial lung disease, cardiac dysfunction, myositis, arthritis, and cutaneous findings.. JDM in the pediatric patient is rare, as is MAS. In patients with complex rheumatologic conditions and lack of response to treatment, it is important to continually assess the patient's clinical status with MAS in mind, as this may change the treatment approach. Without proper recognition of this complication, patients can have a significant delay in diagnosis leading to life-threatening consequences.

Topics: Adolescent; Autoantibodies; Clinical Deterioration; Dermatomyositis; Dose-Response Relationship, Immunologic; Female; Glucocorticoids; Humans; Immunoglobulins, Intravenous; Immunologic Factors; Interferon-Induced Helicase, IFIH1; Macrophage Activation Syndrome; Magnetic Resonance Imaging; Multiple Organ Failure; Mycophenolic Acid; Pulse Therapy, Drug; Treatment Outcome

Pancreatitis is a relatively rare but severe manifestation in systemic lupus erythematosus (SLE) patients. We report a case of a 39-year-old woman with previous SLE diagnose treated with prednisone and mycophenolate mofetil who developed an acute pancreatitis complicated by pancreatic pseudocysts within the context of a severe lupus flare. Elevated serum amylase and computerized tomography confirmed the diagnosis and mechanical obstruction or toxic-metabolic etiologies were ruled out. In the present case, we opted for the clinical surveillance of pancreatic pseudocyst and not perform invasive medical procedures to drainage. A steroid therapy was started in order to achieve SLE and pancreatitis remission, however, it was unable to avoid the development of multiorgan failure and patient died a few days after diagnosis was made.

Topics: Acute Disease; Adult; Amylases; Biomarkers; Drug Therapy, Combination; Fatal Outcome; Female; Glucocorticoids; Humans; Immunosuppressive Agents; Lupus Erythematosus, Systemic; Multiple Organ Failure; Mycophenolic Acid; Pancreatic Pseudocyst; Pancreatitis; Prednisone; Tomography, X-Ray Computed

Pancreatitis following kidney transplantation was first described by Starzl in 1964 [19]. The incidence rate of the disease involving severe complications ranges from 1.2 to 6.8%. The number of risk factors, besides those of the normal population, is increased by a number of other factors, i.e. uremia, disorder of lipid metabolism, polycystic kidney, immunosuppressive drugs, cytomegalovirus infection, etc. The mortality of acute pancreatitis in a kidney transplant patient is, in spite of treatment with the most up-to-date methods, is much higher (53-60%) than that for a non-transplant patient. In the period between 27 June 1991 and 31 December 2000 the number of cadaver kidney transplants performed in the Transplantation Division of the 1st Department of Surgery of the Medical and Health-Science Centre of the University of Debrecen was 349. During this period 9 incidences of acute pancreatitis were found in 8 patients. The frequency of incidence was 2.56%. In the present communication we analyse the prognosis of 9 kidney transplant patients, with special respect to immunosuppression.. One patient was administered Cyclosporin alone, four were given Cyclosporin and Steroids, a further one Cyclosporin, Steroids and Azathioprine, the remaining three were treated with Cyclosporin, steroids and Mycophenolate Mophetil. In six cases out of nine multiorgan insufficiency (kidney, lung, liver) was encountered on presentation, three cases were accompanied by peritonitis. In spite of early jejunal nutrition, intensive therapy, antibiotic treatment, CT monitoring, if needed, necrectomy and oncotomy, three of our patients died from multiorgan insufficiency induced by septico-toxic state (mortality 33.3%). Other six patients recovered.. The mortality rate of acute pancreatitis is much higher in immunosuppressed patients. The role of the etiological factors is not unequivocal in the development of pancreatitis. Nevertheless, all possible risk factors have to be taken into consideration when starting the immunosuppressive treatment of transplant patients and during their follow-up. By optimally adjusting the immunosuppressants we can decrease the risk of pancreatitis, however, the prognosis of the diseases, in agreement with the data in the literature, cannot be considerably improved even with the most up-to-date methods.

Topics: Acute Disease; Adrenal Cortex Hormones; Adult; Azathioprine; Cadaver; Cyclosporine; Female; Humans; Immunosuppressive Agents; Kidney Transplantation; Male; Middle Aged; Multiple Organ Failure; Mycophenolic Acid; Pancreatitis; Peritonitis; Retrospective Studies; Risk Factors