[Diagnostics of Interstitial Lung Diseases - Practical Instructions with a Focus on Rheumatologic Systemic Diseases].

Interstitial lung diseases (ILD) are etiologically heterogeneous with unknown and known causes like rheumatologic systemic diseases differing in their therapeutic and prognostic consequences. In consensus between pulmonologists, rheumatologists, radiologists, and pathologists, we developed practical instructions for ILD diagnosis in rheumatologic systemic diseases, in particular because ILD can present in early stages of rheumatic systemic diseases. ILD diagnosis is based on clinical assessment results including a detailed medical history, physical examination, focused laboratory tests, radiology with a high-resolution computed tomography, lung function, and histopathology also to differentiate it from cardiac and infection associated lung diseases.

Diagnostic yield of axillary artery ultrasound in addition to temporal artery ultrasound for the diagnosis of giant cell arteritis.

Objectives: There are limited data on the additional diagnostic yield of axillary artery ultrasound (axUS) in addition to temporal artery ultrasound (tempUS) for the diagnosis of giant cell arteritis (GCA).

Methods: Retrospective study of consecutive patients with suspected GCA who underwent a standardized axUS and tempUS between 01/2015 and 03/2017. The diagnostic yield of axUS in addition to ultrasound of the temporal arteries with respect to the final clinical diagnosis was assessed, with a positive axUS defined as circumferential, hypoechogenic thickening of the far wall axillary artery intima media thickness (axIMT) ≥1.

Hyperechogenic intimal lesions and wall thickness of the temporal and facial arteries in elderly patients with arterial occlusions of the eye.

Objective: To determine the association of arteriosclerosis, characterised by hyperechogenic intimal lesions (HIL), with wall thickness of the temporal and facial arteries in elderly patients with ocular arterial occlusions.

Methods: Patients suffering from non-arteritic ocular perfusion disorders were included. High-resolution compression sonography (18 MHz) images of the temporal arteries (frontal and parietal branch at the upper margin of the auricle) and facial arteries (at the crossing point of the artery over the mandible) were analysed for the presence of HIL (grade 0: absent; grade 1: moderate; grade 2: severe).

A Diagnostic Algorithm Based on a Simple Clinical Prediction Rule for the Diagnosis of Cranial Giant Cell Arteritis.

Background: Risk stratification based on pre-test probability may improve the diagnostic accuracy of temporal artery high-resolution compression sonography (hrTCS) in the diagnostic workup of cranial giant cell arteritis (cGCA).

Methods: A logistic regression model with candidate items was derived from a cohort of patients with suspected cGCA ( = 87). The diagnostic accuracy of the model was tested in the derivation cohort and in an independent validation cohort ( = 114) by receiver operator characteristics (ROC) analysis.

Temporal artery compression sonography for the diagnosis of giant cell arteritis in elderly patients with acute ocular arterial occlusions.

Objectives: To validate cut-off values of quantitative high-resolution temporal artery compression sonography (TCS) for the diagnosis of cranial GCA (cGCA) in patients with acute arterial ocular occlusions and in an independent control group.

Methods: Consecutive patients who underwent TCS as part of the diagnostic workup of acute arterial ocular occlusions and controls not suffering from ocular ischaemia/systemic vasculitis were included. The diagnostic accuracy of the established TCS cut-off value of maximum temporal artery wall thickness (≥0.

[Update Giant Cell Arteriitis].

Giant cell arteriitis (GCA) is the most common systemic vasculitis in patients ≥  50 years. GCA belongs to the group of large vessel vasculitides. Clinical hallmarks of GCA are persistent localized headache, jaw claudication, limb claudication, symptoms of polymyalgia rheumatica, constitutional symptoms and acute visual symptoms.

Emicizumab in the Treatment of Acquired Haemophilia: A Case Report.

The prognosis of acquired haemophilia A (AHA) is severe and treatment options are limited. Emicizumab is a novel bispecific humanized monoclonal antibody in the treatment of inherited AHA with inhibitors. An 83-year-old AHA patient with congestive heart failure and a high risk for thromboembolic and cardiac events who had initially been treated successfully with steroids and substitution of recombinant B-domain-deleted porcine FVIII developed severe bleeding complications and a secondary increase in inhibitor titres after 4 weeks of treatment.

Ocular ischaemic complications in giant cell arteritis: CHADS2-score predicts risk of permanent visual impairment.

Objectives: To identify independent risk factors for permanent visual loss (PVL) in patients with giant cell arteritis (GCA), with a special focus on sonographic findings of the temporal, carotid and subclavian/axillary arteries, and on established scoring systems of ischaemia risk assessment.

Methods: Consecutive patients with a diagnosis of GCA between 2002 and 2013 were retrospectively identified from a prospectively maintained database. Data on clinical characteristics including ophthalmological findings, laboratory values, and sonographic findings of the temporal, carotid an axillary arteries were extracted.

[Current Recommendations for Diagnostics in Giant Cell Arteritis].

In Giant Cell Arteritis (GCA), a timely diagnosis is required to avoid severe complications such as blindness or structural vascular damage. The heterogeneous symptoms are mainly due to local and systemic inflammatory processes. Diagnostics are based on increased inflammation parameters in the laboratory, imaging, in which color-coded duplex sonography (FKDS), high-resolution magnetic resonance imaging (MRI), computer tomography (CT) or CT angiography (CTA) and F fluorodeoxyglucose-positron emission tomography with CT (FDG-PET-CT) have become established, as well as histopathological findings in temporal artery biopsy.

Assessment of Disease Activity in Takayasu Arteritis: Potential Role of Contrast-Enhanced Ultrasound.

Purpose:  To assess the diagnostic value of intima media thickness measurements and contrast-enhanced ultrasound (CEUS) of the supraaortic arteries in the assessment of disease activity in Takayasu arteritis (TA).

Materials And Methods:  Patients with TA and involvement of the carotid and/or subclavian/axillary arteries underwent CEUS imaging in addition to B-mode and color duplex ultrasound. The maximum IMT (mIMT) was measured and high-resolution CEUS of the most prominently thickened vessel segment was performed.

Diagnostic accuracy of positron emission tomography for assessment of disease activity in large vessel vasculitis.

Aim: To determine the diagnostic yield of F-fluorodeoxyglucose positron emission tomography (PET) in disease activity assessment of large vessel vasculitides (LVV).

Methods: Patients with LVV who had undergone PET (between 2004 and June 2010) or PET co-registered with computed tomography (PET/CT; since June 2010) were identified. Clinical disease activity was assessed using established scoring systems.

Characteristics and treatment of new-onset arthritis after checkpoint inhibitor therapy.

Immune checkpoint inhibitors (ICIs) may cause immune-related adverse events (IRAEs). Characterisation and data on treatment of musculoskeletal IRAEs are scarce. In this cohort study, patients receiving ICI therapy who experienced arthralgia were evaluated for the presence of synovitis.

Three-Dimensional High-Resolution Black-Blood Magnetic Resonance Imaging for Detection of Arteritic Anterior Ischemic Optic Neuropathy in Patients With Giant Cell Arteritis.

Objectives: Arteritic anterior ischemic optic neuropathy (A-AION) caused by inflammatory occlusion of the posterior ciliary arteries is the most common reason for irreversible vision loss in patients with giant cell arteritis. Atypical clinical presentation and negative funduscopy can delay systemic high-dose corticosteroid therapy to prevent impending permanent blindness and involvement of the contralateral eye.The purpose of this study was to assess the diagnostic accuracy of 3-dimensional (3D) high-resolution T1-weighted black-blood magnetic resonance imaging (T1-BB-MRI) for the detection of posterior ciliary artery involvement in patients with giant cell arteritis and funduscopic A-AION.

Clinical value of [18F]FDG-PET/CT and 3D-black-blood 3T-MRI for the diagnosis of large vessel vasculitis and single-organ vasculitis of the aorta.

Background: We aimed to investigate the clinical value of a 3D-T1w turbo-spin-echo (TSE) sequence and [18F]fluorodeoxyglucose positron emission tomography/computed tomography ([18F]FDG-PET/CT) for the diagnosis of active large vessel vasculitis (LVV) and single-organ vasculitis (SOV) of the aorta.

Methods: Twenty-four patients with suspected vasculitis who underwent MRI and PET/CT were retrospectively evaluated. MRI was analyzed for concentric contrast enhancement and wall thickening, and flow artifact intensity (4-point-scales).

Relapses in three patients with Takayasu arteritis under tocilizumab treatment detected by contrast enhanced ultrasound.

Takayasu arteritis (TA) is a rare large vessel vasculitis, affecting the aorta and its major branches, typically in young women. In this case report, we present three cases of young women of Caucasian descent who experienced relapses while under treatment with the monoclonal humanized antibody to the interleukin 6 receptor, tocilizumab. Active vasculitic lesions of the supraaortic (common carotid and axillary) arteries were detected and characterized via high resolution contrast enhanced ultrasound.

B-mode sonography wall thickness assessment of the temporal and axillary arteries for the diagnosis of giant cell arteritis: a cohort study.

Objectives: We aimed to determine the diagnostic accuracy of B-mode compression sonography of the temporal arteries (tempCS) and B-mode sonographic measurement of the axillary artery intima media thickness (axIMT) for the diagnosis of giant cell arteritis (GCA).

Methods: After having established measurement of tempCS and axIMT in our routine diagnostic workup, 92 consecutive patients with a suspected diagnosis of GCA were investigated. Clinical characteristics were recorded and wall thickening of the temporal arteries (tempCS) and axillary arteries (axIMT) was measured (mm).

Gender differences in giant cell arteritis: a case-control study.

Objectives: To determine sex differences in the clinical spectrum and disease pattern of cranial and extracranial giant cell arteritis (GCA).

Methods: Data on 153 consecutive patients with a confirmed diagnosis of GCA between 2002 and 2013 were retrospectively obtained from our database. For every male patient, two age-matched female patients were identified.

Relevance of grade 1 gray-scale ultrasound findings in wrists and small joints to the assessment of subclinical synovitis in rheumatoid arthritis.

Objective: To investigate the clinical relevance of grade 1 findings on gray-scale ultrasound (GSUS) of the joints in patients with rheumatoid arthritis (RA).

Methods: We examined the wrists and small joints of 100 patients with early or established RA and 30 healthy controls, using GSUS and power Doppler ultrasound (PDUS). Independent clinical assessment of all joints for tenderness and swelling according to the European League Against Rheumatism examination technique was performed.

Interleukin 22 serum levels are associated with radiographic progression in rheumatoid arthritis.

Objectives: To study the role of interleukin 22 (IL-22) in rheumatoid arthritis (RA).

Methods: IL-22 serum levels were measured in patients with early, treatment-naive RA (n=49) and in 45 age- and sex-matched healthy individuals as controls. Patients were assessed clinically and radiographically at baseline and followed up for 2 years.

CNS involvement and treatment with interferon-α are independent prognostic factors in Erdheim-Chester disease: a multicenter survival analysis of 53 patients.

Erdheim-Chester disease (ECD) is a rare form of non-Langerhans histiocytosis, with noncodified therapeutic management and high mortality. No treatment has yet been shown to improve survival in these patients. We conducted a multicenter prospective observational cohort study to assess whether extraskeletal manifestations and interferon-α treatment would influence survival in a large cohort of ECD patients.

Role of Th17 cells in human autoimmune arthritis.

Objective: To delineate the role of Th17 cells in the pathogenesis of autoimmune arthritides.

Methods: Th17 cells were analyzed in well-defined homogeneous cohorts of patients with the prototypical autoimmune arthritides rheumatoid arthritis (RA) and psoriatic arthritis (PsA), grouped according to patients who had very early active RA (n = 36; mean disease duration 2.8 months, Disease Activity Score in 28 joints 5.

Standardization of joint examination technique leads to a significant decrease in variability among different examiners.

Objective: To reduce the amount of variability among assessors, we conducted joint examination standardization seminars in conjunction with multicenter clinical trials for patients with rheumatoid arthritis (RA). The examination techniques used were based on the recommendations of the European League Against Rheumatism (EULAR).

Methods: To evaluate the effect of standardization, participants at the seminars examined a given patient with RA before and after they were made familiar with the EULAR examination technique.

Intimate cell conjugate formation and exchange of membrane lipids precede apoptosis induction in target cells during antibody-dependent, granulocyte-mediated cytotoxicity.

Ab-dependent polymorphonuclear granulocyte (PMN)-mediated cytotoxicity may play an important role in the control of malignant diseases. However, little is known as to which particular pathways are used for the killing of malignant cells by PMN. The production of reactive oxygen intermediates (ROI) has been observed to occur during Ab-dependent, cell-mediated cytotoxicity (ADCC).