Debatable aspects of systemic angiitis and autoinfl ammatory diseases

The new data about immune-infl ammatory rheumatic diseases pathogenesis with the participation of the processes of autoimmunity and autoinfl ammation attract attention to the heterogeneity of systemic angiitis, their nomenclature, classifi cation, pathogenesis and the approaches to the therapy. Recommendations of the second International Chapel Hill Consensus Conference (CHCC2012) and EULAR recommendation (2016, 2018) regarding diagnostics and treatment of SA are discussed. The data of 141 patients with various forms of SA and 18 patients with the diseases, which relate to the group of autoinfl ammatory processes as well as the retrospective analysis of 130 patients with the hyperimmunoglobulinemia E are given.

Proposal for a more practical classification of antineutrophil cytoplasmic antibody-associated vasculitis

The nomenclature for antineutrophil cytoplasmic antibody (ANCA)-associated kidney disease has evolved from honorific eponyms to a descriptive-based classification scheme (Chapel Hill Consensus Conference 2012). Microscopic polyangiitis, granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis do not correlate with presentation, response rates and relapse rates as when comparing myeloperoxidase versus leukocyte proteinase 3. Here we discuss the limitations of the currently used classification and propose an alternative, simple classification according to (i) ANCA type and (ii) organ involvement, which provides important clinical information of prognosis and outcomes.

Сutaneous vasculitis: classification, diagnosis and differential diagnosis (part 1)

Cutaneous vasculitis is a heterogeneous group of diseases caused by inflammation of the blood vessel wall of the skin. The variety of cutaneous vasculitis, their clinical similarity, lack of clear diagnostic criteria and common terminology cause an extremely complex process of diagnosis of this group of diseases. A dermatologist is often the first specialist to diagnose vasculitis. Currently there is no single "monodisciplinary" classification of v ascular damage, there is no generally accepted terminology and classification of cutaneous vasculitis. In domestic dermatovenerology, vasculitis is classified according to the morphological elements of the skin rash, and not by etiology and pathogenesis. This makes it difficult for different specialists to understand the diagnosis and maintain continuity in the management of patients. The article presents the nomenclature of vasculitis of the 2012 revised international Chapel Hill consensus conference nomenclature of vasculitides, supplemented by organ-specific cutaneous vasculitis, describes the pathomorphological and clinical manifestations of this group of dermatoses, summarizes information about modern treatment tactics.

Small vessel vasculitis

Small vessel vasculitis is vasculitis affecting predominately small intraparenchymal arteries, arterioles, capillaries, and venules. There are two main types: antineutrophil cytoplasmic antibody associated and immune complex mediated. The ANCA associated vasculitides are discussed in chapter 19.3 IgA vasculitis (IgAV) was formerly known as Henoch Schönlein purpura. The revised nomenclature reflects the importance of IgA vasculitis in pathogenesis. The Chapel Hill Consensus Conference defined IgA vasculitis as ‘vasculitis with IgA1-dominant immune deposits, affecting small vessels (predominantly capillaries, venules, or arterioles)’. IgA vasculitis often involves skin and gut, and frequently causes arthritis. Glomerulonephritis indistinguishable from IgA nephropathy may occur. Its aetiology is unknown, but it frequently occurs after an infection several days to weeks before. The most frequently isolated organism is beta-haemolytic streptococcus. Drugs such as a penicillin, ampicillin, erythromycin, and non-steroidal anti-inflammatory drugs have been reported as precipitating agents. There is an association with HLA-DRB1*01 in Caucasians and there appears to be a familial association.

An Atypical Case of Vasculitis: When ‘Occult’ 18FDG-PET Scan Findings Create a Classification Dilemma

We describe a 66-year old patient with a recurrent ulcer on her right ankle. Biopsy revealed medium-vessel vasculitis consistent with cutaneous polyarteritis nodosa. There were no signs or symptoms suggestive of systemic vasculitis, but a 18FDG-PET scan showed areas of increased uptake around the large arteries and the pelvic and shoulder girdles. These findings suggested polymyalgia rheumatica in the setting of large-vessel vasculitis. This case thus supports the statement from the Chapel-Hill consensus conference that classification of systemic vasculitis by vessel size is based on the vessels predominantly involved, but vessels of other sizes may also be affected.

Primary vasculitides

This chapter describes large, medium, and small vessel primary vasculitides. The 2012 Chapel Hill Consensus Conference nomenclature of vasculitis is introduced, and acts as a framework for more detailed descriptions of polymyalgia rheumatic and giant cell arteritis, polyarteritis nodosa (systemic and cutaneous), granulomatosis with polyangiitis, microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis, Takayasu arteritis, childhood-onset vasculitis, Kawasaki disease, Henoch–Schönlein purpura, and leucocytoclastic vasculitis. Other antineutrophil cytoplasmic antibody-associated vasculitides and primary angiitis of central nervous system in children are also covered. The epidemiology, aetiopathogenesis, classification criteria, presenting features, clinical manifestations, and management of these diseases are presented. In particular, diagnostic criteria for and mimics of polymyalgia rheumatic and giant cell arteritis are discussed in detail to permit rheumatologists and non-rheumatologists to confidently manage them.

Clinical features of kidney involvement in microscopic microscopic polyangiitis

Aim. To evaluate clinical features and outcomes of renal involvement in patients with microscopic polyangiitis (MPA). Materials and methods: We enrolled 99 patients with MPA, diagnosed in accordance with the algorithm of the European Medicines Evaluation Agency (EMEA) and the Chapel Hill consensus conference definition (2012). Serum creatinine (sCr), estimated glomerular filtration rate (eGFR), hematuria and proteinuria were estimated. Frequency of rapidly progressive renal failure (a twofold increase in the sCr level in ≤3 months) was regarded as the clinical equivalent of rapidly progressive glomerulonephritis (RPGN). Results and discussion. Renal involvement was present in 92 (92.9%) patients. RPGN developed in 51 (55,4%) patients. The most common features of kidney involvement were hematuria and subnephrotic proteinuria. Arterial hypertension was revealed in 32 (34.7%) patients and was associated with RPGN (p

Relationship between serologic profile (ANCA type) and clinical features of renal involvement in ANCA-associated vasculitides

Objective. To compare the frequency, clinical features and outcomes of renal involvement in ANCA-associated vasculitides (AAV) in patients with antibodies against proteinase-3 (pr3-ANCA) and myeloperoxidase (MPO-ANCA). Materials and methods. In our retrospective study we enrolled 264 patients, 94 males and 170 females, median age 53 [36; 62] years. Among them 157 were pr3-ANCA positive and 107 were MPO-ANCA positive. AAV was diagnosed according to ACR criteria and Chapel Hill consensus conference definition (2012). Median follow up was 44 [18; 93] months. We assessed baseline BVAS and VDI by the end of the follow up. Serum creatinine (sCr), estimated glomerular filtration rate (eGFR), hematuria and daily proteinuria were estimated. Diagnosis and stage of chronic kidney disease (CKD) and acute kidney injury (AKI) were established according to KDIGO guidelines (2012) and Scientific Society of Russian Nephrologists (2016). Results. Renal involvement was present in 181 (68.6%) patients, and its frequency was similar in pr3-ANCA and MPO-ANCA subgroups. Patients with MPO-ANCA developed rapidly progressive glomerulonephritis and hypertension significantly more often than patients with pr3-ANCA: 50.7% vs 35.6% (p=0.049) and 46.1% vs 29.8% (p=0.029) respectively. At disease onset, median sCr was significantly higher and eGFR was significantly lower in patients with MPO-ANCA (p