High-titer rheumatoid factor seropositivity predicts mediastinal lymphadenopathy and mortality in rheumatoid arthritis-related interstitial lung disease.

Rheumatoid arthritis-related interstitial lung disease (RA-ILD) is a common connective tissue disease-related ILD (CTD-ILD) associated with high morbidity and mortality. Although rheumatoid factor (RF) seropositivity is a risk factor for developing RA-ILD, the relationship between RF seropositivity, mediastinal lymph node (MLN) features, and disease progression is unknown. We aimed to determine if high-titer RF seropositivity predicted MLN features, lung function impairment, and mortality in RA-ILD. In this retrospective cohort study, we identified patients in the University of Chicago ILD registry with RA-ILD. We compared demographic characteristics, serologic data, MLN size, count and location, and pulmonary function over 36 months among patients who had high-titer RF seropositivity (≥ 60 IU/ml) and those who did not. Survival analysis was performed using Cox regression modeling. Amongst 294 patients with CTD-ILD, available chest computed tomography (CT) imaging and serologic data, we identified 70 patients with RA-ILD. Compared to RA-ILD patients with low-titer RF, RA-ILD patients with high-titer RF had lower baseline forced vital capacity (71% vs. 63%; P = 0.045), elevated anti-cyclic citrullinated peptide titer (122 vs. 201; P = 0.001), CT honeycombing (50% vs. 80%; P = 0.008), and higher number of MLN ≥ 10 mm (36% vs. 76%; P = 0.005). Lung function decline over 36 months did not differ between groups. Primary outcomes of death or lung transplant occurred more frequently in the high-titer RF group (HR 2.8; 95% CI 1.1-6.8; P = 0.028). High-titer RF seropositivity was associated with MLN enlargement, CT honeycombing, and decreased transplant-free survival. RF titer may be a useful prognostic marker for stratifying patients by pulmonary disease activity and mortality risk.

TAFRO Syndrome with an Anterior Mediastinal Mass and Lethal Autoantibody-Mediated Thrombocytopenia: An Autopsy Case Report.

TAFRO syndrome, a rare systemic inflammatory disease, can lead to multiorgan failure without appropriate treatment. Although thrombocytopenia is frequently seen in patients with TAFRO syndrome, little is known about its pathogenesis. Moreover, while recent studies have reported the presence of an anterior mediastinal mass in some patients, the pathological status of this remains unclear. Here, we report a case of fatal bleeding in a patient with TAFRO syndrome accompanied by an anterior mediastinal mass. A 55-year-old female was transferred to our hospital with a 2-week history of fever, epistaxis, and dyspnea. Laboratory tests revealed severe thrombocytopenia, computed tomography (CT) showed pleural effusions, and bone marrow biopsy revealed reticulin myelofibrosis. We suspected TAFRO syndrome, but the CT scan showed an anterior mediastinal mass that required a biopsy to exclude malignancy. She soon developed severe hemorrhagic diathesis and died of intracranial hemorrhage despite intensive treatment. She had multiple autoantibodies against platelets, which caused platelet destruction. An autopsy of the mediastinal mass revealed fibrous thymus tissues with infiltration by plasma cells. Our case suggests that thrombocytopenia could be attributed to antibody-mediated destruction and could be lethal. Hence, immediate treatment is imperative in cases of severe thrombocytopenia, even when accompanied by an anterior mediastinal mass.

[Mediastinal fat necrosis]. / Nécrose de la graisse médiastinale.

Epicardial fat necrosis is a rare cause of benign chest pain. Its physiopathological mechanism is unknown. Diagnosis is easily performed through radiological investigations that show a round opacity of fat density limited by a dense pseudo-capsule in the anterior mediastinum, close to the heart.

[Mediastinal parathyroidectomy]. / La paratiroidectomia mediastinica.

In most patients the postoperative persistent or recurrent hyperparathyroidism is sustained by parathyroid tissue localized in the mediastinum. A careful preoperative imaging and intraoperative hormone assays are key to reducing the possibility of disease recurrence.

[Mediastinal adenopathies and presence of anti-cytoplasmic antibodies of diffuse type polynuclear neutrophils]. / Adénopathies médiastinales et présence d'anticorps anti-cytoplasme des polynucléaires neutrophiles de type diffus.

We report a case of sarcoidosis with mediastinal and pulmonary localizations associated with diffuse antineutrophil cytoplasmic antibodies (c-ANCA). This led to discuss the differential diagnosis with vascularitis and the specificity of c-ANCA in Wegener's granulocytosis.

Bilateral pleural effusions in a beta-thalassemia intermedia patient with posterior mediastinal extramedullary hematopoietic masses.

Intractable bilateral exudative pleural effusions developed, following systemic sepsis without pulmonary infection, in a beta-thalassemia intermedia patient with longstanding mediastinal hematopoietic masses. The pleura were not infiltrated by hematopoietic cells. Bilateral talc pleurodesis successfully controlled the effusions.

Does peripheral blood T-lymphocyte population distribution in sarcoidosis provide a prognostic clue?

In its pulmonary form, sarcoidosis generally resolves spontaneously, but it may lead to fibrosis of the lung. The clinical, radiological and functional tests, as well as activity markers such as the serum angiotensin converting enzyme, intrathoracic uptake of 67Gallium and the cytological data provided by bronchoalveolar lavage are only the expressions at any given time of a disease which is constantly progressing and only partly express its evolutive potential. The authors studied the distribution of T-lymphocyte subsets in the peripheral blood and from bronchoalveolar lavage. 32 patients were included in the study. They were suffering from acute or chronic sarcoidosis of the mediastinum and lungs and were divided into 2 groups according to clinical, radiological and pulmonary function criteria; Group A (n = 19) included regressive forms (minimum follow up 2 years) and group B (n = 13) the progressive untreated forms. Lymphopenia with a decrease in the percentage of CD3 cells was found in both groups. The percentage of CD4 cells is significantly lower in group B (28 +/- 11%) than in group A (45 +/- 8%) (p < 0.01) or in the control population (46 +/- 8%) (p < 0.01). The percentage of CD8 cells is higher in group B (30 +/- 8%) than in group A (18 +/- 6%). This results in a CD4/CD8 ratio which is significantly reduced in group B (1 +/- 0.5) when compared with group A (2.72 +/- 0.8) (p < 0.01) and the control group (2.17 +/- 0.8) (p < 0.01), the difference between group A and the controls being minimal.(ABSTRACT TRUNCATED AT 250 WORDS)

Fine needle aspiration of thoracic extramedullary hematopoiesis.

Thoracic manifestations of extramedullary hematopoiesis (EMH) are well known clinically, radiologically and pathologically; it is generally confirmed histologically by open biopsy or postmortem examination. We report a case of EMH diagnosed by percutaneous fine needle aspiration (FNA) in a patient with long-standing, acquired, sideroblastic anemia. The aspirate was very cellular and composed of normal bone marrow elements with an erythroid to myeloid cell ratio of 3 to 1. Megakaryocytes were abundant but constituted less than 1% of the total cell population. This case illustrates that the diagnosis of EMH is possible by FNA and that thoracotomy for diagnostic purposes can be circumvented.

Angiofollicular lymph node hyperplasia (Castleman).

Six personally observed cases of this lesion are reported and the literature is reviewed. One hundred and thirty-four cases have been reported to date. The lesion has occurred in many sites, but is commonest in the thorax (60%), abdomen (11%), neck (14%), and axilla (4%). Ninety per cent of cases are symptomless or have only the pressure symptoms, 10% have systemic signs, namely, fever, raised ESR, anaemia, cured by removal of the tumour. These cases have a slightly different histology. All recorded cases have been benign. Microscopically the lesion is follicular but instead of germinal centres the follicles have one or more thick-walled arteries and often some surrounding histiocytes. Increased numbers of vessels occur between the follicles, whilst sinuses are absent. These vessels have thick, cellular walls like post-capillary venules. Sometimes they become hyaline. The cases with symptoms have true reaction centres as well as a few intrafollicular vessels. Between the follicles there are increased numbers of vessels and, in addition, there may be collections of plasma cells or eosinophils.