ABSTRACT
Vanishing bone disease with multisystemic involvement may mimic systemic autoimmune or autoinflammatory diseases. We present a 19-year-old man who was hospitalized due to chest pain following a progressive osteolysis of the bony thorax. The disease later expanded into the pleura, peritoneum and pericardium in a form of massive chylous polyserositis. The patient also developed thrombosis of multiple central veins, which in turn worsened the chylothorax by increasing the pressure in the thoracic duct. This is the first case of vanishing bone disease complicated by triple chylous effusions and central vein thrombosis.
Subject(s)
Chylothorax/etiology , Osteolysis, Essential/complications , Serositis/etiology , Venous Thrombosis/etiology , Biopsy , Chylothorax/diagnosis , Chylothorax/therapy , Chylous Ascites/etiology , Diagnosis, Differential , Disease Progression , Fatal Outcome , Humans , Lymphoscintigraphy , Male , Osteolysis, Essential/diagnosis , Osteolysis, Essential/therapy , Pericardial Effusion/etiology , Pleural Effusion/etiology , Predictive Value of Tests , Serositis/diagnosis , Serositis/therapy , Tomography, X-Ray Computed , Venous Thrombosis/diagnosis , Venous Thrombosis/therapy , Young AdultABSTRACT
BACKGROUND: The United States Advisory Committee on Immunization Practices recommends administration of the 13-valent pneumococcal conjugate vaccine in series with the 23-valent pneumococcal polysaccharide vaccine for prevention of pneumonia in the elderly. Reports of autoimmune or auto-inflammatory diseases as a result of pneumococcal vaccination, especially pneumococcal conjugate vaccine, are extremely rare. CASE PRESENTATION: We present a case of severe serositis in a 75-year-old Caucasian woman complicated by pericardial and pleural effusions in the setting of recent 13-valent pneumococcal conjugate vaccine vaccination and no other obvious etiology. Our patient required steroid treatment, thoracentesis, chest tube, and pericardial window and subsequently recovered to her baseline. CONCLUSIONS: To the best of our knowledge, no such reaction to the 13-valent pneumococcal conjugate vaccine has previously been documented. Although the benefits of vaccination outweigh the risks, knowledge of this potential side effect can help clinicians in diagnosis and treatment of similar patients.
Subject(s)
Anti-Inflammatory Agents/therapeutic use , Pericardial Effusion/chemically induced , Pleural Effusion/chemically induced , Pneumococcal Vaccines/adverse effects , Prednisone/therapeutic use , Serositis/chemically induced , Vaccination/adverse effects , Aged , Drainage , Female , Humans , Pericardial Effusion/immunology , Pericardial Effusion/therapy , Pleural Effusion/immunology , Pleural Effusion/therapy , Pneumococcal Vaccines/administration & dosage , Serositis/immunology , Serositis/therapy , Thoracentesis , Treatment Outcome , Vaccines, ConjugateABSTRACT
Serositis is a rare manifestation of chronic GvHD (cGvHD). No risk factors or laboratory changes associated with this syndrome have been recognized to date, and outcomes have not been described in a large series. We searched our institutional database for patients undergoing allogeneic hematopoietic cell transplant identified as having serositis or pericarditis. Laboratory studies from prior to diagnosis, at diagnosis and post diagnosis of serositis, as well as outcomes from invasive procedures were included. Twenty patients met criteria for cGvHD-associated serositis, and all but three patients had a prior diagnosis of cGvHD. Fifteen were male, and the complication occurred in the setting of immunosuppressant taper in 12 cases. Ten patients required invasive interventions, including pericardial window or stripping. A significant increase in blood monocytes and decrease in serum albumin were identified at diagnosis compared with pre-diagnosis. Out of 20 patients, 17 were treated with steroids, with 12 demonstrating a complete response. These data suggest that cGvHD-associated serositis occurs mainly in the setting of treated as opposed to de novo cGvHD and biomarkers associated with the syndrome include a decrease in albumin and an increase in absolute monocyte count. Outcome data from larger series are required to better understand the optimal management of this rare complication.
Subject(s)
Graft vs Host Disease/diagnosis , Graft vs Host Disease/therapy , Pericarditis/diagnosis , Pericarditis/therapy , Serositis/diagnosis , Serositis/therapy , Adult , Aged , Allografts , Chronic Disease , Female , Graft vs Host Disease/blood , Hematologic Neoplasms/blood , Hematologic Neoplasms/therapy , Humans , Male , Middle Aged , Pericarditis/blood , Serositis/bloodABSTRACT
Multicentric Castleman's disease (MCD) is a polyclonal lymphoproliferative disorder that manifests as marked hyper-γ-globulinemia, severe inflammation, anemia, and thrombocytosis. Recently, Takai et al. reported a new disease concept, TAFRO syndrome, named from thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly. Furthermore, Kojima et al. reported Japanese MCD cases with effusion and thrombocytopenia (Castleman-Kojima disease). Here, we report two cases of MCD associated with marked pleural effusion, ascites, and thrombocytopenia, and discuss the independence of the TAFRO syndrome (Castleman-Kojima disease). Case 1: A 57-year-old woman had fever, anemia, anasarca, and some small cervical lymphadenopathy. Although she had been administered steroid therapy, and full-coverage antibiotics, her general condition, including fever, systemic inflammation, and anasarca, deteriorated steadily. We administered chemotherapy [CHOEP (cyclophosphamide, doxorubicin, vincristine, etoposide, and prednisolone) regimen], but despite a transient improvement, she died due to septic shock. Case 2: A 73-year-old man with a history of aplastic anemia and remission presented with fever, severe inflammation, and anasarca. Prednisolone was administered (15 mg daily), and his hyperinflammation once improved. Nevertheless, his general condition, including pleural effusion and ascites, worsened, and C-reactive protein and interleukin-6 levels showed marked increases. The patient died due to multiorgan failure. Cases of TAFRO syndrome (Castleman-Kojima disease) are still rare. Therefore, it is necessary to conduct multicenter clinical surveys including similar cases, such as ours, to reach a consensus regarding diagnostic criteria, therapeutic strategy, and pathophysiological etiology for this syndrome.
Subject(s)
Castleman Disease/diagnosis , Serositis/pathology , Thrombocytopenia/pathology , Aged , Asian People , Castleman Disease/blood , Castleman Disease/pathology , Castleman Disease/therapy , Female , Humans , Male , Middle Aged , Serositis/therapy , Thrombocytopenia/therapyABSTRACT
We report a patient who developed multiple serositis during chronic graft-versus-host disease (GVHD) after allogeneic bone marrow transplantation from a non-inherited maternal antigen (NIMA) -complementary sibling donor. The patient was a 9-year-old boy with myelodysplastic syndrome, who urgently underwent bone marrow transplantation from his NIMA-complementary HLA two-locus-mismatched sister following graft failure of cord blood transplantation. Engraftment was successfully confirmed and no acute GVHD developed. After withdrawal of tacrolimus to prevent recurrent viral infection, he developed pleural effusion, ascites and edema approximately 6 months after transplantation. His clinical symptoms were resolved by methylprednisolone pulse therapy, but he subsequently progressed to develop pericardial effusion, pneumothorax and truncal panniculitis. Pleural and pericardial effusion contained numerous lymphocytes, which gradually subsided with continuous drainage. His symptoms were thereafter controlled by the addition of mycophenolate mofetil (MMF) administration, and his current performance status is almost perfect by the administration of prednisolone (5 mg/day) and MMF at 6 years after transplantation. Although multiple serositis associated with GVHD is known to have a poor prognosis, the multiple symptoms of this patient improved gradually, probably owing to a lack of acute GVHD and the effect of MMF.
Subject(s)
Bone Marrow Transplantation , Graft vs Host Disease/therapy , Myelodysplastic Syndromes/therapy , Serositis/therapy , Siblings , Tissue Donors , Adolescent , Child , Chronic Disease , Drainage , Female , Graft vs Host Disease/etiology , HLA Antigens , Histocompatibility , Humans , Immunosuppressive Agents/administration & dosage , Male , Mycophenolic Acid/administration & dosage , Mycophenolic Acid/analogs & derivatives , Serositis/etiology , Treatment OutcomeABSTRACT
Although the benefits of clozapine have been well demonstrated in resistant schizophrenia, the frequency of adverse events is of particular concern: up to 76% of patients to whom clozapine was prescribed experienced an adverse event, with a discontinuation rate of 17%. In addition to its major clinical side effect, agranulocytosis, clozapine is reported to induce inflammatory syndromes with polyserositis. Apart from sparse case reports, no study has yet addressed this particularly interesting issue. With the aim of improving the outcome of clozapine-treated patients, we undertook a review of the literature to characterize the clinical features of clozapine-induced serositis, its pathophysiology, and to propose strategies of clinical management.
Subject(s)
Antipsychotic Agents/adverse effects , Clozapine/adverse effects , Schizophrenia/drug therapy , Serositis/chemically induced , Adult , Female , Humans , Male , Schizophrenia/physiopathology , Serositis/physiopathology , Serositis/therapy , Withholding TreatmentABSTRACT
BACKGROUND: Serosal appendicitis is a histopathological diagnosis of an inflammatory reaction on the surface of the appendix caused by an extra appendiceal source of inflammation. OBJECTIVE AND METHODS: A retrospective review of a pathology database in a district general hospital identified patients with serosal appendicitis and a preoperative diagnosis of appendicitis. Two groups emerged: patients with serosal appendicitis secondary to a known cause of intra-abdominal inflammation and those in whom the cause remained unknown. The groups were compared with respect to postoperative complications, referral to outpatient clinics, admissions and procedures by case-note analysis. RESULTS: The incidence of serosal appendicitis was 0.01% (19/1379); 17 women; mean age 31.63 (10.49) years. Lanz incision was used in 16 patients, midline laparotomy in two and laparoscopy in one. A preoperative ultrasound scan was carried out in 5 of 19 patients. The cause of serosal appendicitis was confirmed in eight patients (intraoperatively or postoperatively) and remained unknown in 11 patients. The groups were comparable with respect to age, sex and mean follow-up. No significant difference was observed between them with respect to the above parameters. CONCLUSIONS: No further investigation is necessary in patients who undergo an appendicectomy but in whom the pathological diagnosis is serosal appendicitis. However, considering the patient demographics and the fact that patients with serosal appendicitis have probably undergone an unnecessary appendicectomy, the proportion of patients who underwent ultrasound scanning and diagnostic laparoscopy was small.
Subject(s)
Appendicitis , Serositis , Abdominal Pain/etiology , Adolescent , Adult , Appendectomy/methods , Appendectomy/statistics & numerical data , Appendicitis/etiology , Appendicitis/surgery , Female , Humans , Male , Middle Aged , Retrospective Studies , Serositis/etiology , Serositis/therapySubject(s)
Anti-Inflammatory Agents/therapeutic use , Lupus Erythematosus, Systemic/complications , Prednisolone/therapeutic use , Pyloric Stenosis/complications , Adult , Cystitis/therapy , Female , Gastroscopy , Humans , Peritonitis/therapy , Pyloric Stenosis/therapy , Serositis/therapy , Tomography, X-Ray ComputedABSTRACT
Es frecuente encontrar que los pacientes con lupus eritematoso sistémico (LES) presenten, durante el transcurso de la enfermedad, afección de las membranas serosas, principalmente la pleura y el pericardio. La poliserositis es, sin embargo, bastante rara como modo de presentación del LES, más aun cuando hay efección del peritoneo. Presentamos un grupo de cinco pacientes jóvenes con derrame pleural, pericárdico y ascitis como manifestación de una poliserositis lúpica temprana. Todos tuvieron compromiso sistémico y afección renal, que requirieron un tratamiento agresivo. Dos pacientes fallecieron (uno por complicaciones infecciosas y otro por causas no esclarecidas). La poliserositis temprana en el LES quizás constituya un marcador clínico de actividad de la enfermedad
Subject(s)
Humans , Lupus Erythematosus, Systemic/complications , Serositis/etiology , Lupus Erythematosus, Systemic/therapy , Serositis/diagnosis , Serositis/therapyABSTRACT
Se presenta una experiencia con 6 casos de seromas quísticos periprotésicos entre 1974 y 1986. El tiempo de aparición fue entre los 3 meses y 3 años. Los materiales protésicos utilizados fueron en 4 dacron "knitted", en PTFE y en 1 vena umbilical. Se recomienda el tratamiento conservador con punciones e inyección de antibióticos dejando la conducta de resección para la recidiva o la infección (AU)
Subject(s)
Middle Aged , Aged , Humans , Vascular Surgical Procedures , Blood Vessel Prosthesis/adverse effects , Serositis/therapy , Postoperative Complications , DrainageABSTRACT
Se presenta una experiencia con 6 casos de seromas quísticos periprotésicos entre 1974 y 1986. El tiempo de aparición fue entre los 3 meses y 3 años. Los materiales protésicos utilizados fueron en 4 dacron "knitted", en PTFE y en 1 vena umbilical. Se recomienda el tratamiento conservador con punciones e inyección de antibióticos dejando la conducta de resección para la recidiva o la infección
