[Hughes-Stovin syndrome: a life-threatening manifestation of Behçet's syndrome].

Hughes-Stovin syndrome (HSS) is a systemic inflammatory condition of unknown origin that is considered to be part of the Behçet's syndrome (BS) spectrum. Recurrent venous thrombosis and superficial thrombophlebitis in combination with bilateral pulmonary artery aneurysms (PAA) represent the hallmark of HSS. The diagnostic evaluation includes computed tomography pulmonary angiography to detect signs of pulmonary vasculitis.

[Granulomatous vasculitides and vasculitides with extravascular granulomatosis].

Vasculitides are inflammatory diseases of blood vessels caused by autoimmune or infectious processes, which are associated with alterations and destruction of the vascular wall. From a histopathological point of view, granulomatous vasculitides can be distinguished from necrotizing vasculitides with respect to the pattern of inflammation. Granulomatous vasculitides are characterized by intramural, predominantly lymphohistiocytic infiltrates with the formation of giant cells.

[Accreditation in pathology and neuropathology : Paths and pitfalls].

There are many good reasons for accreditation in pathology or neuropathology as per DIN EN ISO/IEC 17020, regardless of the size and range of services of the facility. Only accreditation - in contrast to certification - also confirms professional competence. This article describes how to establish a quality management system that conforms to standards as effectively as possible and how to maintain it, involve staff, and avoid common pitfalls.

[Cocaine-induced vasculitis and mimics of vasculitis].

Cocaine is a psychotropic tropane alkaloid and stimulant drug. Nasal insufflation of cocaine powder is a common route of administration. In Germany, cocaine is frequently adulterated with levamisole, an anthelminthic drug with immunomodulatory effects.

[Update on etiopathogenesis of small vessel vasculitis].

Small vessel vasculitis is characterized by a necrotizing inflammation of the vessel wall predominantly with involvement of small intraparenchymal arteries, arterioles, capillaries and venules. Medium-sized and occasionally large vessels can also be involved. Antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (granulomatosis with polyangiitis, microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis) are differentiated from immune complex vasculitides based on immunopathological and serological aspects.

The Joint Vasculitis Registry in German-speaking countries (GeVas) - a prospective, multicenter registry for the follow-up of long-term outcomes in vasculitis.

Background: Vasculitides comprise a group of rare diseases which affect less than 5 in 10.000 individuals. Most types of vasculitis can become organ- and life-threatening and are characterized by chronicity, high morbidity and relapses, altogether resulting in significant morbidity and mortality.

GPA-Induced Granulomatous Endocarditis Mimicking a Thrombotic Mitral Valve Stenosis.

We present the case of a patient with granulomatous endocarditis of the mitral valve leading to severe valve stenosis caused by granulomatosis with polyangiitis. Endocarditis is a rare complication of granulomatosis with polyangiitis that may be misdiagnosed as infectious endocarditis or, as in our case, thrombotic lesions. ().

Granulomatous Inflammation in ANCA-Associated Vasculitis.

ANCA-associated vasculitis (AAV) comprises granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). While systemic vasculitis is a hallmark of all AAV, GPA is characterized by extravascular granulomatous inflammation, preferentially affecting the respiratory tract. The mechanisms underlying the emergence of neutrophilic microabscesses; the appearance of multinucleated giant cells; and subsequent granuloma formation, finally leading to scarred or destroyed tissue in GPA, are still incompletely understood.

[Pathogenesis of large vessel vasculitides].

Large vessel vasculitides comprise two distinct entities, giant cell arteritis (GCA) and Takayasu arteritis (TAK). GCA is the most common vasculitis in central Europe, becoming manifested at an age over 50 years. In contrast, the much rarer TAK affects almost exclusively young adults and mostly women.

[Cutaneous vasculitis and vasculopathy : Differential diagnosis in biopsies of the lower extremities].

The skin is one of the most frequently involved organs in primary systemic and secondary vasculitis; moreover, a vasculitis can occur as single organ vasculitis, limited to the skin. For most types of vasculitis, the lower extremities constitute common sites with clinical symptoms of palpable purpura, nodules, and ulcers. In histopathology of cutaneous vasculitis, it is of utmost importance to correctly identify the vessel types involved and to discriminate between vasculitic vessel damage, unspecific reactive vessel changes, vascular occlusive diseases, noninflammatory purpura, or perivascular infiltrates due to other inflammatory skin diseases.

Giant retroperitoneal liposarcoma: A case report and literature review.

Retroperitoneal liposarcomas are a heterogeneous group of mesenchymal tumours that have a wide spectrum of histological subtypes and vague clinical presentations. Herein, we present the case of a 75-year-old man with anorexia, weight loss of 7 kg, and a growing abdominal circumference within a span of 6 weeks. Computed tomography of the abdomen and pelvis showed a large mass that filled almost the entire abdominal cavity.

In situ detection of PR3-ANCA B cells and alterations in the variable region of immunoglobulin genes support a role of inflamed tissue in the emergence of auto-reactivity in granulomatosis with polyangiitis.

Circulating anti-neutrophilic cytoplasmic autoantibodies targeting proteinase 3 (PR3-ANCA) are a diagnostic and pathogenic hallmark of granulomatosis with polyangiitis (GPA). It is, however, incompletely understood if inflamed tissue supports presence or emergence of PR3-ANCA B cells. In search of such cells in inflamed tissue of GPA, immunofluorescence staining for IgG and a common PR3-ANCA idiotype (5/7 Id) was undertaken.

Successful reconstruction of an ocular defect resulting from granulomatosis with polyangiitis, following treatment with rituximab.

Purpose: To report a unique case of orbital inflammatory disease which was ultimately diagnosed as granulomatosis with polyangitis (GPA) and thus successfully treated.

Observation: A 47 year-old man presented with a rapidly progressive necrotic soft tissue mass within the medial antero-superior aspect of the right eyelid and orbit. He also had transient retinal vasculitis in the left.

Pathogenetic and Clinical Aspects of Anti-Neutrophil Cytoplasmic Autoantibody-Associated Vasculitides.

Anti-neutrophil cytoplasmic autoantibodies (ANCA) targeting proteinase 3 (PR3) and myeloperoxidase expressed by innate immune cells (neutrophils and monocytes) are salient diagnostic and pathogenic features of small vessel vasculitis, comprising granulomatosis with polyangiitis (GPA), microscopic polyangiitis, and eosinophilic GPA. Genetic studies suggest that ANCA-associated vasculitides (AAV) constitute separate diseases, which share common immunological and pathological features, but are otherwise heterogeneous. The successful therapeutic use of anti-CD20 antibodies emphasizes the prominent role of ANCA and possibly other autoantibodies in the pathogenesis of AAV.

[Granulomatous reaction pattern of the skin : Interstitial granulomatous dermatitis - lymphoma - vasculitis].

A particular diagnostic situation in the classification of a granulomatous dermatitis results when no circumscribed granulomas but instead a diffuse interstitial histiocytic inflammatory infiltrate, a granulomatous vasculitis or a neoplastic lymphocytic infiltrate is found. Interstitial granulomatous dermatitis was originally described in patients with arthritis. Later, it was recognized that there are also associations with other usually immunological diseases.

[Granulomatosis with polyangiitis].

Granulomatosis with polyangiitis (GPA) is a potentially life-threatening, rare disease. The etiology is unknown. GPA is histomorphologically characterized by extravascular necrotizing granulomatous inflammation and a systemic necrotizing vasculitis of small to medium-sized vessels.

[Non-infectious granulomatous inflammation: Focus on the lungs and skin].

Whereas a granulomatous reaction represents a physiologically useful immune defense mechanism against many infections, in autoimmune diseases granuloma formation and the concomitant inflammatory mechanisms may provoke a potentially organ-threatening reaction. Morphologically, several defined sub-types of granuloma have long been known, e.g.

[Polyarteritis nodosa: differential diagnostics and therapy].

Polyarteritis nodosa (PAN) is a necrotizing vasculitis of medium size arteries that may affect various organs. The clinical appearance is very variable. The most common manifestations are of the skin, the peripheral nervous system presenting as mononeuritis multiplex and the mesenteric and renal blood vessels due to the development of stenoses and small aneurysms.

[Vasculitis: New nomenclature of the Chapel Hill consensus conference 2012].

Within the last years, many advances have been made in the understanding of the etiopathology of vasculitis as well as of different disease courses. The revised 2012 Chapel Hill consensus conference (CHCC) nomenclature reflects current knowledge on the etiopathology in addition to the descriptive principles of vessel size and types of inflammation. The anti-neutrophil cytoplasmic antibody (ANCA)-associated forms of vasculitis have been separated as a group, as opposed to immune complex small vessel vasculitis.