Extensive, circumferential, epidural spinal cord compression by IgG4-related pachymeningitis.

A rare case of IgG4-related pachymeningitis is presented. The manuscript describes the multidisciplinary treatment of a rare entity in an exceptional location, prompting emergent surgical treatment. However, the correct intraoperative hypothesis led to a minimal surgical approach with fewer risks and good disease control.

Spectrum of histiocytic neoplasms associated with diverse haematological malignancies bearing the same oncogenic mutation.

Histiocytic disorders are a spectrum of rare diseases characterised by the accumulation of macrophage-, dendritic cell-, or monocyte-differentiated cells in various tissues and organs. The discovery of recurrent genetic alterations in many of these histiocytoses has led to their recognition as clonal neoplastic diseases. Moreover, the identification of the same somatic mutation in histiocytic lesions and peripheral blood and/or bone marrow cells from histiocytosis patients has provided evidence for systemic histiocytic neoplasms to originate from haematopoietic stem/progenitor cells (HSPCs).

Immunopathogenesis of granulomas in chronic autoinflammatory diseases.

Granulomas are clusters of immune cells. These structures can be formed in reaction to infection and display signs of necrosis, such as in tuberculosis. Alternatively, in several immune disorders, such as sarcoidosis, Crohn's disease and common variable immunodeficiency, non-caseating granulomas are formed without an obvious infectious trigger.

Chronic signs of memory B cell activation in patients with Behçet's disease are partially restored by anti-tumour necrosis factor treatment.

Objectives: Behçet's disease (BD), an auto-inflammatory vasculitis with oro-genital ulcerations, skin lesions and uveitis, is regarded as T cell mediated. A successful trial with rituximab suggests an additive role for B cells in the pathogenesis. Therefore, we studied B cell abnormalities in BD patients and the effect of TNF-blocking therapy.

B-Cell Dysregulation in Crohn's Disease Is Partially Restored with Infliximab Therapy.

Background: B-cell depletion can improve a variety of chronic inflammatory diseases, but does not appear beneficial for patients with Crohn's disease.

Objective: To elucidate the involvement of B cells in Crohn's disease, we here performed an 'in depth' analysis of intestinal and blood B-cells in this chronic inflammatory disease.

Methods: Patients with Crohn's disease were recruited to study B-cell infiltrates in intestinal biopsies (n = 5), serum immunoglobulin levels and the phenotype and molecular characteristics of blood B-cell subsets (n = 21).

Bone Mineral Density in Sjögren Syndrome Patients with and Without Distal Renal Tubular Acidosis.

Primary Sjögren's syndrome (pSS) can be complicated by distal renal tubular acidosis (dRTA), which may contribute to low bone mineral density (BMD). Our objective was to evaluate BMD in pSS patients with and without dRTA as compared with healthy controls. BMD of lumbar spine (LS) and femoral neck (FN) was measured in 54 pSS patients and 162 healthy age- and sex-matched controls by dual-energy X-ray absorptiometry (DXA).

Cytokine and viral load kinetics in human herpesvirus 8-associated multicentric Castleman's disease complicated by hemophagocytic lymphohistiocytosis.

Human herpes virus 8 (HHV-8)-associated secondary hemophagocytic lymphohistiocytosis is a rare but critical immuno-hematological entity in immunocompetent patients. Establishing a diagnosis is challenging as is the monitoring of disease activity and therapeutic effects. We report a case of a HHV-8-associated hemophagocytic lymphohistiocytosis in a HIV-negative adult patient with multicentric Castleman's disease.

Somatostatin receptor scintigraphy patterns in patients with sarcoidosis.

Purpose: Sarcoidosis is a multisystem granulomatous disorder, most frequently involving the lungs, skin, or eyes. Somatostatin receptor scintigraphy (SRS) can visualize sarcoid granulomas through binding of a radionuclide-coupled somatostatin analog to somatostatin receptors that are expressed in sarcoidosis. Uptake and patterns on SRS were studied and correlated to clinical and conventional findings.

Prevalence of distal renal tubular acidosis in primary Sjögren's syndrome.

Objectives: Our objectives were to analyse the prevalence of distal renal tubular acidosis (dRTA) in primary SS (pSS) and to compare a novel urinary acidification test with furosemide and fludrocortisone (FF) with the gold standard ammonium chloride (NH4Cl) to detect dRTA.

Methods: Urinary acidification was assessed in 57 pSS patients using NH4Cl and FF. A urinary acidification defect was defined as an inability to reach a urinary pH of <5.

Calcium and vitamin D in sarcoidosis: is supplementation safe?

Granulomas in sarcoidosis express high levels of 1α-hydroxylase, an enzyme that catalyzes the hydroxylation of 25-OH vitamin D to its active form, 1,25(OH)2 vitamin D. Overproduction of 1α-hydroxylase is held responsible for the development of hypercalcemia in sarcoidosis patients. Corticosteroids are used as first-line treatment in organ-threatening sarcoidosis.

Everything you need to know about distal renal tubular acidosis in autoimmune disease.

Renal acid-base homeostasis is a complex process, effectuated by bicarbonate reabsorption and acid secretion. Impairment of urinary acidification is called renal tubular acidosis (RTA). Distal renal tubular acidosis (dRTA) is the most common form of the RTA syndromes.

Utility of contrast-enhanced ultrasound for the assessment of the carotid artery wall in patients with Takayasu or giant cell arteritis.

Aims: Carotid contrast-enhanced ultrasound (CEUS) was recently proposed for the evaluation of large-vessel vasculitides (LVV), particularly to assess vascularization within the vessel wall. The aim of this pilot study was to evaluate the potential of carotid colour Doppler ultrasound (CDUS) and CEUS in patients with LVV.

Methods And Results: This prospective study included seven patients (mean age 48 ± 14 years, all females) with established LVV (Takayasu arteritis or giant cell arteritis).

[Diagnosis and treatment of uveitis; not restricted to the ophthalmologist].

Visual symptoms due to uveitis involve a wide range of possible causes. Familiarity with its clinical manifestations, referral indications and treatment strategies is required for the optimal use of current therapeutic options. Uveitis can be caused by infectious and non-infectious factors, resulting in differing prognoses and treatments.

Perigranuloma localization and abnormal maturation of B cells: emerging key players in sarcoidosis?

Rationale: Recent observations of abnormal immunoglobulin responses and case reports describing successful B-cell ablative therapy suggest involvement of B cells in the pathogenesis of sarcoidosis.

Objectives: To investigate how abnormal B-cell maturation and function in patients with sarcoidosis contribute to disease.

Methods: Patients with sarcoidosis (n = 32) were included for detailed analysis by immunohistochemistry of tissue, flow cytometry of blood B-cell subsets, and serum immunoglobulin levels.

[Colchicine has no negative effect on fertility and pregnancy].

Objective: To inventorise the possible development of infertility and pregnancy complications in patients with Familial Mediterranean Fever (FMF), on treatment with colchicine.

Design: Systematic review.

Method: PubMed was searched for articles in English, describing the effects of colchicine on fertility and pregnancy in animals or humans.

[Sarcoidosis: changing insights in therapy].

Sarcoidosis is a granulomatous disease of unknown etiology. Standard treatment with immune suppressants such as glucocorticoids is started when vital organ function is threatened. Biotechnology has resulted in new treatments ('biologicals'), in particular monoclonal antibodies, that may be effective in the treatment of sarcoidosis.