Síndrome de Churg-Strauss, Aneurismas Femorais Venosos e Tromboembolismo Pulmonar

OBJECTIVE: Venous aneurysms of the lower limbs are rare. When they involve the deep venous system, they can be associated with deep venous thrombosis and pulmonary embolism. The authors report the case of a 63-year-old women evaluated by duplex imaging and venography, in the context of a pulmonary embolism, and detected bilateral large aneurysms of the femoral veins (5 cm in the right femoral vein and 4 cm in the left femoral vein). The aneurysms were located at the bifurcation of the common femoral vein with the superficial and profound veins. METHOD: The right aneurysm was partially thrombosed. The patient was asymptomatic in the lower-limb extremities. After a period of anticoagulation, the authors proceeded to surgically excise the right aneurysm and replaced the venous system using a polytetrafluoroethylene (PTFE) graft with the construction of an a-v fistula. RESULT: The patient had no complications in the postoperative period and her 8-month follow-up examination revealed no symptoms, not even leg edema. The duplex scan showed patency of the graft. She continued with oral anticoagulation. The histologic examination revealed an eosinophilic inflammatory reaction of the vein wall, compatible with the diagnosis of Churg-Strauss syndrome. CONCLUSION: The authors conclude that they have probably described the first case in the international literature of a venous aneurysm with a Churg-Strauss syndrome and pulmonary embolism.

Dilated Cardiomyopathy. New Therapeutic Approach

Inflammatory reaction has been associated with dilated cardiomyopathy. In this context, cardiac autoantibodies and inflammatory cell infiltration have been studied during the last two decades towards the understanding of their origin and the underlying pathogenic mechanisms. Recent research has increasingly focused on the development of an etiological therapeutic approach. Immunoadsorption has shown to improve clinical, echocardiographic, haemodynamic and laboratory parameters in patients with inflammatory dilated cardiomyopathy. In this article we review recent literature concerning this subject, including classification, pathophysiological mechanisms and therapy.

Polymerase Chain Reaction Screening for Fungemia and/or Invasive Fungal Infections in Patients with Hematologic Malignancies

INTRODUCTION: Invasive fungal infections (IFIs) are a life-threatening complication in patients with hematologic malignancies, mainly in acute leukemia patients, following chemotherapy. IFI incidence is increasing, and associated mortality remains high due to unreliable diagnosis. Antifungal drugs are often limited by inadequate antimicrobial spectrum and side effects. Thus, the detection of circulating fungal DNA has been advocated as a rapid, more sensitive diagnostic tool. PATIENTS AND METHODS: Between June 01 and January 03, weekly blood samples (1,311) were screened from 193 patients undergoing intensive myelosuppressive or immunosuppressive therapy. IFI cases were classified according to European Organization for Research and Treatment of Cancer/Mycoses Study Group criteria. Fungal DNA was extracted from whole blood and amplified using polymerase chain reaction (PCR) published primers that bind to the conserved regions of the fungal 18S rRNA gene sequence. In our study, two or more consecutive positive samples were always associated with fungal disease. RESULTS: PCR screening predicted the development of IFI to be 17 days (median). This test had a specificity of 91.1% and a sensitivity of 75%. IFI incidence was 7.8%. DISCUSSION: Therefore, our results confirm the potential usefulness of PCR serial screening and the clinical applicability in everyday routine. PCR screening offers a noninvasive repeatable aid to the diagnosis of IFI.

Leprosy and Kaposi Sarcoma Presenting as an Immune Reconstitution Inflammatory Syndrome in a Patient with AIDS

The simultaneous presence of infectious organisms within cutaneous lesions of Kaposi sarcoma in persons with AIDS has been demonstrated. We describe a patient with concurrent leprosy and Kaposi sarcoma presenting as an immune reconstitution inflammatory syndrome in the setting of AIDS.

Anti-Tumor Necrosis Factor Alpha-Induced Drug Eruptions: One Patient, More Than a Pattern

Background: Tumor necrosis factor alpha (TNFα) antagonists are effective in treating several immune-inflammatory diseases, including psoriasis and inflammatory bowel disease. The paradoxical and unpredictable induction of psoriasis and psoriasiform skin lesions is a recognized adverse event, although of unclear aetiology. However, histological analysis of these eruptions remains insufficient, yet suggesting that some might constitute a new pattern of adverse drug reaction, rather than true psoriasis. Case report: The authors report the case of a 43-year-old woman with severe recalcitrant Crohn disease who started treatment with infliximab. There was also a personal history of mild plaque psoriasis without clinical expression for the past eight years. She developed a heterogeneous cutaneous eruption of psoriasiform morphology with pustules and crusts after the third infliximab infusion. The histopathological diagnosis was of a Sweet-like dermatosis. The patient was successfully treated with cyclosporine in association with both topical corticosteroid and vitamin D3 analogue. Three weeks after switching to adalimumab a new psoriasiform eruption was observed, histologically compatible with a psoriasiform drug eruption. Despite this, and considering the beneficial effect on the inflammatory bowel disease, it was decided to maintain treatment with adalimumab and to treat through with topicals, with progressive control of skin disease. Discussion: Not much is known about the pathogenesis of psoriasiform eruptions induced by biological therapies, but genetic predisposition and Koebner phenomenon may contribute to it. Histopathology can add new facets to the comprehension of psoriasiform reactions. In fact, histopathologic patterns of such skin lesions appear to be varied, in a clear asymmetry with clinical findings. Conclusion: The sequential identification in the same patient of two clinical and histopathologic patterns of drug reaction to TNFα antagonists is rare. Additionally, to the authors’ knowledge, there is only one other description in literature of a TNFα antagonist-induced Sweet-like dermatosis, emphasizing the singularity of this case report.

Curcumin Inhibits Gastric Inflammation Induced by Helicobacter Pylori Infection in a Mouse Model

Helicobacter pylori (H. pylori) infection triggers a sequence of gastric alterations starting with an inflammation of the gastric mucosa that, in some cases, evolves to gastric cancer. Efficient vaccination has not been achieved, thus it is essential to find alternative therapies, particularly in the nutritional field. The current study evaluated whether curcumin could attenuate inflammation of the gastric mucosa due to H. pylori infection. Twenty-eight C57BL/6 mice, were inoculated with the H. pylori SS1 strain; ten non-infected mice were used as controls. H. pylori infection in live mice was followed-up using a modified 13C-Urea Breath Test (13C-UBT) and quantitative real-time polymerase chain reaction (PCR). Histologically confirmed, gastritis was observed in 42% of infected non-treated mice at both 6 and 18 weeks post-infection. These mice showed an up-regulation of the expression of inflammatory cytokines and chemokines, as well as of toll-like receptors (TLRs) and MyD88, at both time points. Treatment with curcumin decreased the expression of all these mediators. No inflammation was observed by histology in this group. Curcumin treatment exerted a significant anti-inflammatory effect in H. pylori-infected mucosa, pointing to the promising role of a nutritional approach in the prevention of H. pylori induced deleterious inflammation while the eradication or prevention of colonization by effective vaccine is not available.

Drug-Induced Anaphylaxis Survey in Portuguese Allergy Departments

Background and Objective: Drug-induced anaphylaxis is an unpredictable and potentially fatal adverse drug reaction. The aim of this study was to identify the causes of drug-induced anaphylaxis in Portugal. Methods: During a 4-year period a nationwide notification system for anaphylaxis was implemented, with voluntary reporting by allergists. Data on 313 patients with drug anaphylaxis were received and reviewed. Statistical analysis included distribution tests and multiple logistic regression analysis to investigate significance, regression coefficients, and marginal effects. Results: The mean (SD) age of the patients was 43.8 (17.4) years, and 8.3% were younger than 18 years. The female to male ratio was 2:1. The main culprits were nonsteroidal anti-inflammatory drugs (NSAIDs) (47.9% of cases), antibiotics (35.5%), and anesthetic agents (6.1%). There was a predominance of mucocutaneous symptoms (92.2%), followed by respiratory symptoms (80.4%) and cardiovascular symptoms (49.0%). Patients with NSAID-induced anaphylaxis showed a tendency towards respiratory and mucocutaneous manifestations. We found no significant associations between age, sex, or atopy and type of drug. Anaphylaxis recurrence was observed in 25.6% of cases, and the risk was higher when NSAIDs were involved. Conclusions: NSAIDs were the most common cause of anaphylaxis in this study and were also associated with a higher rate of recurrence. We stress the need for better therapeutic management and prevention of recurring episodes of drug-induced anaphylaxis.

Inflammatory Pseudotumor of the Urinary Bladder

We report a case of an inflammatory pseudotumor of the urinary bladder in a 31 year-old woman. She presented at the emergency room with low abdominal pain and urinary symptoms. Abdominal ultrasound, computed tomography and magnetic resonance imaging were performed and revealed asymmetric thickening of the urinary bladder wall. Cystoscopy with urinary cytology revealed a benign nature of the process. The patient underwent partial cystectomy and the pathologic examination of the specimen revealed an inflammatory pseudotumor. We reviewed the clinical, imaging and pathological features of the inflammatory pseudotumor of the urinary bladder and discussed its differential diagnosis.

Implant Site Nexplanon Reaction?

Nexplanon (Schering-Plough Limited/Merck Sharp & Dohme Limited (MSD)) is a long active reversible contraceptive method that provides effective contraception for 3 years. It consists of a single, flexible, rod-shaped implant, containing 68 mg etonogestrel. It is 4 cm long, consists of an ethylene vinyl acetate copolymer, a non-absorbable material, and also contains 15 mg of barium sulfate, which makes it visible by X-ray. We describe a case of a 39-year-old woman who experienced a local reaction to the barium sulfate in Nexplanon. She was given medical treatment, but only the removal of the implant resolved the symptoms. After removal there was gradual improvement and 72 h later the patient was asymptomatic. Allergic reaction to barium sulfate is extremely rare: until now, there have only been two cases associated with Nexplanon described in the literature.

Giant Angiolymphoid Hyperplasia with Eosinophilia on the Chest

Angiolymphoid hyperplasia with eosinophilia is a rare vascular proliferation characterized by single or multiple purplish, brownish papules and subcutaneous nodules, sometimes associated with pain or pruritus. This rare benign process occurs with a female predominance. Approximately 85% of the lesions occur in the skin of the head and neck; most of them are around the ear or on the forehead or scalp. Whether angiolymphoid hyperplasia with eosinophilia represents a benign neoplasm or an unusual reaction to varied stimuli, including trauma, the etiology remains unclear. Histopathologically, the lesions consist of a proliferation of blood vessels of variable size lined by large epithelioid endothelial cells and a variable inflammatory infiltrate of lymphocytes and eosinophils, sometimes with lymphoid follicle formation. The lesion is benign but may be persistent and is difficult to eradicate. We report on a case of a 58-year-old Caucasian man who presented a purplish pink dome-shaped tumor of size up to 8 cm in diameter located on the chest. We emphasize this case considering the unusual dimensions of the lesion (8 cm diameter) and the atypical location on the chest.

Recommendations for Vaccination in Adult Patients with Systemic Inflammatory Rheumatic Diseases from the Portuguese Society of Rheumatology

Serious infections are a major cause of morbidity and mortality in systemic inflammatory rheumatic disease (SIRD) patients. Although vaccination may prevent numerous infections, vaccination uptake rates are low in this group of patients. OBJECTIVES: To develop evidence-based recommendations for vaccination in SIRD patients. METHODS: We searched MEDLINE (until 31 October 2014) and EMBASE (until 14 December 2014) databases, as well as the ACR and EULAR congress abstracts (2011-2014). Patients with any systemic inflammatory rheumatic disease were included and all vaccines were considered. Any safety and efficacy outcomes were admitted. Search results were submitted to title and abstract selection, followed by detailed review of suitable studies. Data were subsequently pooled according to the type of vaccine and the SIRD considered. Results were presented and discussed by a multidisciplinary panel and systematic literature review (SLR)-derived recommendations were voted according to the Delphi method. The level of agreement among rheumatologists was assessed using an online survey. RESULTS: Eight general and seven vaccine-specific recommendations were formulated. Briefly, immunization status should routinely be assessed in all SIRD patients. The National Vaccination Program should be followed and some additional vaccines are recommended. To maximize the efficacy of vaccination, vaccines should preferably be administered 4 weeks before starting immunosuppression or, if possible when disease activity is controlled. Non-live vaccines are safe in SIRD, including immunosuppressed patients. The safety of live attenuated vaccines in immunosuppressed patients deserves further ascertainment, but might be considered in particular situations. DISCUSSION: The present recommendations combine scientific evidence with the multidisciplinary expertise of our taskforce panel and attained desirable agreement among Portuguese rheumatologists. Vaccination recommendations need to be updated on a regular basis, as more scientific data regarding vaccination efficacy and safety, emergent infectious threats, new vaccines as well as new immunomodulatory therapies become available.