Refractory systemic lupus erythematosus with chylous effusion successfully treated with sirolimus: a case report and literature review.

Chylous effusion is a rare manifestation of systemic lupus erythematosus (SLE). When it does occur in SLE, it is generally well treated with standard pharmacologic or surgical measures. We present a decade of management in a case of SLE with lung affliction and development of refractory bilateral chylous effusion and pulmonary arterial hypertension (PAH). In the first years, the patient was treated under a Sjogren syndrome diagnose. After few years, her respiratory condition worsened due to chylous effusion and PAH. Immunosuppression therapy (methylprednisolone) was reintroduced, and vasodilator therapy commenced. With this, her cardiac function remained stable, but respiratory function continuously worsened despite several therapy trials with different combinations of immunosuppressant (glucocorticoids, resochin, cyclophosphamide and mycophenolate mofetil). On top of pleural effusion worsening, the patient developed ascites and severe hypoalbuminaemia. Even though albumin loss was stabilized with monthly octreotide applications, the patient remained respiratory insufficient and in need of continuous oxygen therapy. At that point, we decided to introduce sirolimus on top of glucocorticoids and mycophenolate mofetil therapy. Her clinical status, radiological finding, and lung function gradually improved and she became respiratory sufficient at rest. The patient remains in our follow-up and has been stable on given therapy for over 3 years despite overcoming a severe COVID-19 pneumonia in 2021. This case adds to the body of evidence of sirolimus effectiveness in patients with refractory systemic lupus and is, to our best knowledge, the first case to report its successful application in a patient with SLE and refractory chylous effusion.

New Insight in the Q

Virtual Compton scattering on the proton has been investigated at three yet unexplored values of the four-momentum transfer Q^{2}: 0.10, 0.20, and 0.45 GeV^{2}, at the Mainz Microtron. Fits performed using either the low-energy theorem or dispersion relations allowed the extraction of the structure functions P_{LL}-P_{TT}/ε and P_{LT}, as well as the electric and magnetic generalized polarizabilities α_{E1}(Q^{2}) and ß_{M1}(Q^{2}). These new results show a smooth and rapid falloff of α_{E1}(Q^{2}), in contrast to previous measurements at Q^{2}=0.33 GeV^{2}, and provide for the first time a precise mapping of ß_{M1}(Q^{2}) in the low-Q^{2} region.

Early onset of nontuberculous mycobacterial pulmonary disease contributes to the lethal outcome in lung transplant recipients: report of two cases and review of the literature.

Lung transplant (LuTx) recipients represent a population at risk of nontuberculous mycobacterial pulmonary disease (NTM-PD). Yet the risk factors, the timing of NTM-PD after transplantation, and the association with allograft dysfunction all remain poorly defined. We report 2 cases of early-onset NTM-PD and review the literature, focusing on NTM-PD in LuTx recipients not colonized with NTM prior to transplantation. In addition, we summarize the main characteristics and differences between early- and late-onset disease.

Measurement of the neutron electric to magnetic form factor ratio at Q2=1.58 GeV2 using the reaction 3He[over →](e[over →],e'n)pp.

A measurement of beam helicity asymmetries in the reaction 3He[over →](e[over →],e'n)pp is performed at the Mainz Microtron in quasielastic kinematics to determine the electric to magnetic form factor ratio of the neutron GEn/GMn at a four-momentum transfer Q2=1.58 GeV2. Longitudinally polarized electrons are scattered on a highly polarized 3He gas target. The scattered electrons are detected with a high-resolution magnetic spectrometer, and the ejected neutrons are detected with a dedicated neutron detector composed of scintillator bars. To reduce systematic errors, data are taken for four different target polarization orientations allowing the determination of GEn/GMn from a double ratio. We find µnGEn/GMn=0.250±0.058(stat)±0.017(syst).

Literature Highlights.

Literature Highlights is a digest of notable papers recently published in the leading respiratory journals, allowing our readers to stay up-to-date with research advances. Coverage in this issue includes Vitamin D supplementation to prevent TB infection; network models of TB dynamics through enhanced data collection linked to active case-finding; hydrocortisone use for severe community-acquired pneumonia; and low-cost air quality sensors and individual exposure levels.

Literature Highlights.

Literature Highlights is a digest of notable papers recently published in the leading respiratory journals. Coverage includes shorter regimen for TB treatment; mapping the geographical evolution of TB incidence; diagnostic packages for active case finding for TB; TB burden estimation in settings with high levels of HIV; and digital approaches for TB treatment adherence.

Literature Highlights.

Literature Highlights is a digest of notable papers recently published in the leading respiratory journals. Coverage includes clinical trials to investigate the diagnostic and clinical effect of trial of antibiotics on TB; a Phase 3 trial to assess if glucocorticoids decrease mortality among patients with pneumonia; a Phase 2 trial on pretomanid use for treating drug-susceptible TB; contact investigation for TB in China; and post-TB sequelae after TB treatment in children.

High-precision determination of the electric and magnetic form factors of the proton.

New precise results of a measurement of the elastic electron-proton scattering cross section performed at the Mainz Microtron MAMI are presented. About 1400 cross sections were measured with negative four-momentum transfers squared up to Q² = 1 (GeV/c)² with statistical errors below 0.2%. The electric and magnetic form factors of the proton were extracted by fits of a large variety of form factor models directly to the cross sections. The form factors show some features at the scale of the pion cloud. The charge and magnetic radii are determined to be ½ = 0.879(5)stat(4)syst(2)model(4)group fm and ½ = 0.777(13)stat(9)syst(5)model(2)group fm.

From Peruvian mummies to living humans: first case of pulmonary tuberculosis caused by Mycobacterium pinnipedii.

The zoonotic potential of Mycobacterium tuberculosis complex species is well known. However, M. pinnipedii, the causative agent of tuberculosis (TB) predominantly in seals and sea lions, has never been isolated from a respiratory specimen in humans. Here we describe the first known human case of pulmonary TB caused by M. pinnipedii in a 79-year-old female patient with rheumatoid arthritis and chronic respiratory disease. The epidemiological data did not explain where the patient was exposed to M. pinnipedii, thus leaving the source of transmission unknown.

Lung Abscess: An Early Complication of Lung Transplantation in a Patient with Cystic Fibrosis.

A 22-year-old woman with cystic fibrosis (CF) developed lung abscess, as a rare complication caused by multidrug-resistant (MDR) Acinetobacter baumannii infection, after lung transplantation (LT). After 6 months of long-term antibiotic therapy, the abscess was successfully eliminated. In reviewed published literature, no previous report was found describing this kind of complication caused by MDR A. baumannii in post-LT patient with CF. In our experience, lung abscess in LT recipients with CF can be successfully treated with prolonged antibiotic therapy.

Benign lipoblastoma in the mandible.

Large benign tumors and cysts of the mandible cause such massive bulging in the soft tissue of the cheek that one is tempted to try to find an external approach to the tumor. In our experience an oral approach has proven to be sufficient and advantageous in most cases of benign mandibular tumors even when they were very large. This is demonstrated in an unusual case of lipoblastoma of the ascending mandibular ramus. It is the first endosteal case of this rare tumor in the literature.

Malignant transformation of a nasopharyngeal angiofibroma.

The malignant transformation of a nasopharyngeal angiofibroma is a rare occurrence. This report describes the development of a fibrosarcoma in a patient over a 20-year period after four surgical procedures and two courses of radiation therapy to control a nasopharyngeal angiofibroma. A review of the literature revealed similar descriptions of this problem. Radiation likely plays a major role in causing the malignant change. An initial complete surgical excision can prevent recurrence of a nasopharyngeal angiofibroma as well as sarcomatous transformation. With the availability of surgical procedures that can completely and safely resect almost all nasopharyngeal angiofibromas, radiation therapy can be avoided.

The histopathological and clinical features of irradiated and nonirradiated temporal paragangliomas.

This study was undertaken to determine the effects of radiation on temporal paragangliomas. The histological features were assessed in a blind fashion from 20 surgical specimens obtained from 20 patients, half of whom received radiotherapy. All patients had progressive temporal paragangliomas and were selected on a random basis for this study. The derived data clearly identify the unpredictable response of these tumors to radiation and supports our contention that surgery is the preferred form of treatment for temporal paragangliomas even after radiation therapy. Analysis of clinical histories reveals that previous radiation therapy is associated with a greater operative blood loss and a longer mean postoperative hospital stay due to delayed healing. Despite this, there was no mortality or serious morbidity from surgery.

The surgical management of extensive nasopharyngeal angiofibromas with the infratemporal fossa approach.

Large juvenile nasopharyngeal angiofibromas are a therapeutic challenge because of their relation to major vasculature and cranial nerves at the base of the skull, and their propensity for recurrence. A classification scheme based on the growth pattern of this tumor is proposed to help the surgeon choose a procedure to access this lesion. This report describes the results obtained with the surgical removal of large (class III and IV) nasopharyngeal angiofibromas through the infratemporal fossa approach. Fourteen patients were cured and one individual developed a recurrence which was totally removed at a second procedure. Surgical morbidity was minimal and there was no mortality. Radiation therapy was necessary in only one patient who had tumor infiltration of the cavernous sinus.

Histology of synthetic ossicular prostheses.

Thirty-three synthetic middle ear prostheses removed after two to 99 months of implantation were examined histologically. All prostheses were infiltrated by fibroblasts, capillaries, collagen fibrils, and multinucleate foreign body giant cells. Although some of the giant cells contained tiny particles of the prosthetic material, there was no evidence of erosion or engulfment of larger particles of the prosthesis or structural dissolution of the prosthesis. New histologic findings were aseptic necrosis and focal calcification in the interior of some of the prostheses.

Intraoral malignant melanotic schwannoma. Ultrastructural evidence for melanogenesis by Schwann's cells.

Clinical and light and electron microscopic findings of one case of malignant melanotic schwannoma of the oral cavity are presented. The tumor recurred four times and developed submandibular metastasis. At autopsy, 24 months after manifestation of the initial symptoms, a hematogenous metastatic nodule was present in the liver. Peripheral melanotic schwannomas show a more malignant behavior than intraspinal examples. The Schwann's cell character of the individual tumor cells is demonstrated ultrastructurally by the presence of a prominent basal lamina, desmosomelike junctions between interdigitated elongated cell processes, and melanosomes in all stages of formation. These findings support the concept that neoplastic human Schwann's cells are capable of melanogenesis.

Lesions of Rathke's duct: another indication for the infratemporal fossa approach.

Rathke's duct is a midline structure, arising from the primitive stomodeum and extending upward to the hypophysis. The duct, which normally involutes in utero, may persist on occasion. This most frequently results in intracranial disease. Occasionally, disease may become manifest in the extracranial skull base. We present three such cases: cystic craniopharyngioma, Rathke's cleft cyst, and a persistent cerebrospinal fluid rhinorrhea caused by a patent craniopharyngeal canal. Each lesion involved the clivus and the parasphenoid region. Surgical management of such lesions requires not only extirpation of disease, but also the ability to recognize and preserve the many important regional structures. The procedure that best allows for the wide exposure necessary to accomplish these goals is the infratemporal fossa approach to the lateral skull base.

So called "fibro-osseous lesions" of tumorous origin. Biology confronts terminology.

The terms "fibro-osteo-cemental lesions" or "ossifying" and "cementifying fibromas" are confusing and should be discarded and replaced by clear and distinct terminology based on clinically, radiologically and histopathologically observable and reproducible characteristics. Histogenetic features and biological potential rather than tumour tissue products alone should be regarded as the basis for nomenclature. A classification of "fibro-osseous lesions" and three tumorous entities which have been defined by the terms "periodontoma", "psammous desmo-osteoblastoma" and "trabecular desmo-osteoblastoma" are presented and illustrated. These proposed entities go some way to bridge the gap between clinical diagnosis on the one hand and morphological and descriptive diagnosis on the other.

Diagnostic accuracy of TMJ arthroscopy in correlation to histological findings.

From 1987-1993, 356 arthroscopic examinations of the temporomandibular joint were performed on 295 patients. During 69 examinations, biopsies were obtained to correlate arthroscopic findings with histology. In the overall group, histology confirmed in 78.9% the arthroscopic findings. Correlation was better in joints with degenerative changes (81.5%) than in those with a synovitic/hyperaemic appearance (61.5%). Chondroid metaplasia, detritus synovitis and synovial chondromatosis were additional diagnoses given by histological examination. In 11 joints, open arthrotomy was performed after arthroscopy with biopsy was carried out. The excised tissue was also investigated microscopically and correlates to the biopsy-result. Because no different pathological changes were found, it is concluded that biopsies performed during arthroscopy of the temporomandibular joint are representative for histological investigation. An additional perforation for introducing the biopsy forceps is not necessary, because results obtained with different techniques appeared to be equally accurate.