OBJECTIVE: Granulomatosis with polyangiitis (GPA) is an ANCA-associated vasculitis. The 2022 ACR/EULAR-endorsed classification criteria for GPA was derived using data only from adult patients. We aimed to assess the performance of the ACR/EULAR classification criteria for GPA in paediatric patients and compare it with the EULAR/Pediatric Rheumatology International Trials Organization (PRINTO)/Pediatric Rheumatology European Society (PReS)-endorsed Ankara 2008 criteria for GPA. METHODS: Retrospective data of paediatric patients with GPA in 20 centres from 9 countries were evaluated. The diagnosis of GPA was made according to the expert opinion. The sensitivity, specificity, positive predictive value, and negative predictive value of the criteria sets were evaluated. RESULTS: The study included 77 patients with GPA and 108 controls [IgA vasculitis (n = 44), Takayasu's arteritis (n = 20), microscopic polyangiitis (n = 16), polyarteritis nodosa (n = 14), Behçet's disease (n = 12), eosinophilic granulomatosis with polyangiitis (n = 1) and Cogan's syndrome (n = 1)] with a median age of 17.8 and 15.2 years, respectively. Among patients with GPA, constitutional symptoms (85.7%) and ENT involvement (79.2%) were the most common presentations. In the GPA group, 73 patients fulfilled the Ankara 2008 criteria and 69 the ACR/EULAR classification criteria. Sensitivities of the Ankara 2008 criteria and the ACR/EULAR classification criteria were 94.8% and 89.6%, while specificities were 95.3% and 96.3%, respectively. No significant difference was found between sensitivities and specificities of both classification criteria (P = 0.229 and P = 0.733, respectively). CONCLUSION: In children, both the ACR/EULAR and EULAR/PRINTO/PReS Ankara 2008 classification criteria for GPA perform well and similarly.

• MeSH
• Behcet Syndrome classification   diagnosis   MeSH
• Churg-Strauss Syndrome diagnosis   classification   MeSH
• Child MeSH
• Granulomatosis with Polyangiitis * classification   diagnosis   MeSH
• IgA Vasculitis diagnosis   classification   MeSH
• Humans MeSH
• Microscopic Polyangiitis classification   diagnosis   MeSH
• Adolescent MeSH
• Polyarteritis Nodosa classification   diagnosis   MeSH
• Predictive Value of Tests MeSH
• Child, Preschool MeSH
• Retrospective Studies MeSH
• Rheumatology standards   MeSH
• Sensitivity and Specificity * MeSH
• Takayasu Arteritis * classification   diagnosis   MeSH
• Check Tag
• Child MeSH
• Humans MeSH
• Adolescent MeSH
• Male MeSH
• Child, Preschool MeSH
• Female MeSH
• Publication type
• Journal Article MeSH
• Multicenter Study MeSH
• Comparative Study MeSH
• Geographicals
• Europe MeSH

BACKGROUND: Nephritis is a common manifestation of IgA vasculitis and is morphologically indistinguishable from IgA nephropathy. While MEST-C scores are predictive of kidney outcomes in IgA nephropathy, their value in IgA vasculitis nephritis has not been investigated in large multiethnic cohorts. METHODS: Biopsies from 262 children and 99 adults with IgA vasculitis nephritis ( N =361) from 23 centers in North America, Europe, and Asia were independently scored by three pathologists. MEST-C scores were assessed for correlation with eGFR/proteinuria at biopsy. Because most patients ( N =309, 86%) received immunosuppression, risk factors for outcomes were evaluated in this group using latent class mixed models to identify classes of eGFR trajectories over a median follow-up of 2.7 years (interquartile range, 1.2-5.1). Clinical and histologic parameters associated with each class were determined using logistic regression. RESULTS: M, E, T, and C scores were correlated with either eGFR or proteinuria at biopsy. Two classes were identified by latent class mixed model, one with initial improvement in eGFR followed by a late decline (class 1, N =91) and another with stable eGFR (class 2, N =218). Class 1 was associated with a higher risk of an established kidney outcome (time to ≥30% decline in eGFR or kidney failure; hazard ratio, 5.84; 95% confidence interval, 2.37 to 14.4). Among MEST-C scores, only E1 was associated with class 1 by multivariable analysis. Other factors associated with class 1 were age 18 years and younger, male sex, lower eGFR at biopsy, and extrarenal noncutaneous disease. Fibrous crescents without active changes were associated with class 2. CONCLUSIONS: Kidney outcome in patients with biopsied IgA vasculitis nephritis treated with immunosuppression was determined by clinical risk factors and endocapillary hypercellularity (E1) and fibrous crescents, which are features that are not part of the International Study of Diseases of Children classification.

• MeSH
• Biopsy MeSH
• Child MeSH
• Adult MeSH
• Glomerular Filtration Rate MeSH
• Glomerulonephritis, IGA * complications   drug therapy   pathology   MeSH
• IgA Vasculitis * complications   drug therapy   pathology   MeSH
• Kidney pathology   MeSH
• Humans MeSH
• Adolescent MeSH
• Nephritis * complications   MeSH
• Proteinuria etiology   MeSH
• Retrospective Studies MeSH
• Check Tag
• Child MeSH
• Adult MeSH
• Humans MeSH
• Adolescent MeSH
• Male MeSH
• Publication type
• Journal Article MeSH

Přehledná část článku je věnována diferenciální diagnostice artralgií a artritid s důrazem na infekční etiologii. Jsou shrnuty základní přístupy k diagnostice artritid u dětských revmatických onemocnění v ambulanci dětské revmatologie a také možnosti, jak k tomuto postupu mohou přispět praktičtí lékaři pro děti a dorost (PLDD). Přinášíme kazuistiky dětských pacientů s artritidou provázející revmatická onemocnění. Artritida může být prvním projevem juvenilní idiopatické artritidy (JIA), systémového lupus erythematodes (SLE), IgA vaskulitidy a mnoha dalších revmatických nemocí, nebo se vyvine v jejich průběhu. Časná diagnóza je klíčem k úspěšné léčbě.

An article is focused on differential diagnosis of arthralgias and arthritides with stress on infectious etiology. We review basic attitudes in diagnostics of pediatric rheumatic diseases and how general practitioners might contribute. Case reports of children with arthritis as a symptom of pediatric rheumatic diseases are described. The arthritis might be the first symptom of juvenile idiopathic arthritis (JIA), systemic lupus erythematosus (SLE), IgA vasculitis and many other rheumatic diseases, or it develops during the disease course. An early diagnosis is a clue to successful therapy.

• MeSH
• Arthralgia * etiology   MeSH
• Arthritis * drug therapy   classification   physiopathology   MeSH
• Diagnosis, Differential MeSH
• Child MeSH
• IgA Vasculitis diagnosis   drug therapy   complications   MeSH
• Arthritis, Infectious etiology   drug therapy   pathology   MeSH
• Arthritis, Juvenile diagnosis   drug therapy   physiopathology   MeSH
• Humans MeSH
• Adolescent MeSH
• Lupus Nephritis diagnosis   drug therapy   complications   MeSH
• Lupus Erythematosus, Systemic drug therapy   complications   MeSH
• Check Tag
• Child MeSH
• Humans MeSH
• Adolescent MeSH
• Female MeSH
• Publication type
• Case Reports MeSH
• Research Support, Non-U.S. Gov't MeSH

Vaskulitidy představují heterogenní skupinu onemocnění různorodé etiologie primárně postihující zánětlivým procesem cévní stěnu, který vede k uzávěru cévního lumen. V důsledku ischemie tkáně zásobené postiženou cévou vznikají klinické stavy s častými kožními projevy. Vaskulitidy mohou být systémové, či postihující pouze jeden orgán, mohou být primárně kožní, nebo kožní postižení se rozvíjí sekundárně, např. u vaskulitid doprovázející systémová onemocnění pojiva. Základní klasifikace představuje anatomicko-patologické dělení dle kalibru postižené cévy zahrnující arterie, vény, kapiláry či postkapilární venuly. Rozlišujeme vaskulitidy velkých cév, které představují aortu a její kmenové větve, vaskulitidy středních cév představují cévy svalové, podkožní a hlubší dermis. Vaskulitidy malých cév postihující kůži se obecně označují jako leukocytoklastické vaskulitidy a postihují cévy střední a papilární části dermis. Jedním z nejčastějších kožních projevů vaskulitid je palpovatelná purpura, která může být přítomna u vaskulitid malých i středních cév. Vzhledem k rozmanitému klinickému obrazu je nutné klást důraz na mezioborovou spolupráci. Autor uvádí současný stav klasifikace vaskulitid a stručný popis jednotlivých klinických jednotek.

Vasculitides are a heterogeneous group of diseases of various etiologies. The inflammatory process primarily affects the vessel wall, which leads to the occlusion of vascular lumen. As a result of ischemia of the tissue supplied by the affected vessel, clinical conditions with frequent skin lesions arise. Vasculitis may be systemic or affect only one organ. It may be primarily cutaneous, or the skin condition develops secondarily, e.g. due to vasculitis associated with systemic connective tissue disease. The basic classification represents the anatomical pathological differentiaton according to the size of the affected vessel, including arteries, veins, capillaries or postcapillary venules. We distinguish vasculitides of large vessels, representing the aorta and its stem branches, vasculitides of middle vessels represented by vessels in muscles, subcutaneous tissue and deeper dermis. Small vessels vasculitis affecting the skin is generally referred to as leukocytoclastic vasculitis and affects the vessels of the middle and papillary parts of the dermis. One of the most common cutaneous manifestations of vasculitis is palpable purpura, which may be manifestation of small and medium vessel vasculitis. Due to the diverse clinical picture interdisciplinary approach is necessary. The author presents the current state of classification of vasculitides and a brief description of individual vasculitic diseases.

• MeSH
• Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis physiopathology   therapy   MeSH
• IgA Vasculitis physiopathology   therapy   MeSH
• Vasculitis, Leukocytoclastic, Cutaneous physiopathology   therapy   MeSH
• Humans MeSH
• Vasculitis * classification   physiopathology   therapy   MeSH
• Check Tag
• Humans MeSH
• Publication type
• Review MeSH

• MeSH
• Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis pathology   MeSH
• IgA Vasculitis pathology   MeSH
• Mucocutaneous Lymph Node Syndrome pathology   MeSH
• Skin Diseases, Vascular pathology   MeSH
• Vasculitis, Leukocytoclastic, Cutaneous pathology   MeSH
• Skin Manifestations MeSH
• Giant Cell Arteritis pathology   MeSH
• Polyarteritis Nodosa pathology   MeSH
• Takayasu Arteritis pathology   MeSH
• Vasculitis classification   complications   MeSH

• MeSH
• Arthralgia diagnosis   etiology   therapy   MeSH
• Autoimmune Diseases diagnosis   classification   MeSH
• Child MeSH
• IgA Vasculitis MeSH
• Arthritis, Juvenile diagnosis   drug therapy   complications   MeSH
• Mucocutaneous Lymph Node Syndrome diagnosis   drug therapy   MeSH
• Humans MeSH
• Adolescent MeSH
• Musculoskeletal Diseases * diagnosis   classification   therapy   MeSH
• Lupus Erythematosus, Systemic MeSH
• Vaccination MeSH
• Age Factors MeSH
• Check Tag
• Child MeSH
• Humans MeSH
• Adolescent MeSH
• Publication type
• Review MeSH

• MeSH
• Child MeSH
• Edema etiology   MeSH
• IgA Vasculitis * diagnosis   therapy   MeSH
• Treatment Outcome MeSH
• Check Tag
• Child MeSH
• Female MeSH
• Publication type
• Case Reports MeSH
• Research Support, Non-U.S. Gov't MeSH

Cryoglobulins are immunoglobulins that undergo reversible precipitation at cold temperatures. Monoclonal type-I cryoglobulinaemia is the least frequent and is associated to hematological diseases such as multiple myeloma, Waldenström's macroglobulinaemia, chronic lymphocytic leukaemia and lymphoma. We describe the case of a 60-year-old female patient, who suffered from burning pain in her feet for ten months before her admission. The patient presented intermittent distal cyanosis that progressed to digital ischaemia. She also reported paresthesia in her hands, difficulty in writing, and a 26-kg-weight loss. At the physical examination, it was identified livedo reticularis, palpable purpura, and painful ecchymotic lesions in her calves and feet. Moreover, peripheral pulses were palpable and symmetrical. It was observed an atrophy of the right first dorsal interosseous and both extensor digitorum brevis, as well as a distal bilateral apalesthesia and allodynia. Both Achilles reflexes were absent. Laboratory tests revealed anemia, high erythrosedimentation rate and C-reactive protein. Serum protein electrophoresis showed a monoclonal IgG-Kappa gammopathy. The results also evidenced the presence of Bence-Jones proteinuria. The bone marrow biopsy revealed less than 10% of plasma cells, and skin biopsy informed leukocytoclastic vasculitis. The patient was treated with high-dose intravenous steroids and cyclophosphamide. The treatment showed that the skin lesions had improved, pain disappeared and motor deficit stopped its progression.

• MeSH
• Adult MeSH
• IgA Vasculitis * MeSH
• Vasculitis, Leukocytoclastic, Cutaneous * MeSH
• Cryoglobulinemia * complications   diagnosis   MeSH
• Middle Aged MeSH
• Humans MeSH
• Multiple Myeloma * MeSH
• Monoclonal Gammopathy of Undetermined Significance * MeSH
• Check Tag
• Adult MeSH
• Middle Aged MeSH
• Humans MeSH
• Female MeSH
• Publication type
• Journal Article MeSH
• Case Reports MeSH

OBJECTIVES: IgA vasculitis (IgAV, formerly known as Henoch-Schönlein purpura) is the most common cause of systemic vasculitis in childhood. To date, there are no internationally agreed, evidence-based guidelines concerning the appropriate diagnosis and treatment of IgAV in children. Accordingly, treatment regimens differ widely. The European initiative SHARE (Single Hub and Access point for paediatric Rheumatology in Europe) aims to optimize care for children with rheumatic diseases. The aim therefore was to provide internationally agreed consensus recommendations for diagnosis and treatment for children with IgAV. METHODS: Recommendations were developed by a consensus process in accordance with the EULAR standard operating procedures. An extensive systematic literature review was performed, and evidence-based recommendations were extrapolated from the included papers. These were evaluated by a panel of 16 international experts via online surveys and subsequent consensus meeting, using nominal group technique. Recommendations were accepted when ⩾80% of experts agreed. RESULTS: In total, 7 recommendations for diagnosis and 19 for treatment of paediatric IgAV were accepted. Diagnostic recommendations included: appropriate use of skin and renal biopsy, renal work-up and imaging. Treatment recommendations included: the importance of appropriate analgesia and angiotensin-converting enzyme inhibitor use and non-renal indications for CS use, as well as a structured approach to treating IgAV nephritis, including appropriate use of CS and second-line agents in mild, moderate and severe disease along with use of angiotensin-converting enzyme inhibitors and maintenance therapy. CONCLUSION: The SHARE initiative provides international, evidence-based recommendations for the diagnosis and treatment of IgAV that will facilitate improvement and uniformity of care.

• MeSH
• Analgesia methods   MeSH
• Biopsy MeSH
• Child MeSH
• Gastrointestinal Diseases diagnosis   etiology   MeSH
• Glucocorticoids therapeutic use   MeSH
• Glomerulonephritis, IGA diagnosis   drug therapy   etiology   pathology   MeSH
• IgA Vasculitis complications   diagnosis   drug therapy   pathology   MeSH
• Immunoglobulin A analysis   MeSH
• Angiotensin-Converting Enzyme Inhibitors therapeutic use   MeSH
• Skin pathology   MeSH
• Kidney pathology   MeSH
• Humans MeSH
• Evidence-Based Medicine methods   MeSH
• Severity of Illness Index MeSH
• Check Tag
• Child MeSH
• Humans MeSH
• Publication type
• Journal Article MeSH
• Consensus Development Conference MeSH
• Research Support, Non-U.S. Gov't MeSH
• Practice Guideline MeSH

BACKGROUND: Henoch-Schönlein purpura (HSP) is a systemic disorder characterized by leukocytoclastic vasculitis involving the capillaries and by the deposition of IgA immune complexes. An association between HSP and atypical bacteria is uncommon in children. METHODS AND RESULTS: Here we report three cases of children, aged 5, 4 and 16 years, who were diagnosed with HSP associated with Mycoplasma pneumoniae or Chlamydia pneumoniae infection. In all presented cases, persistent cutaneous manifestations and abdominal pain were resistant to antibiotics and corticosteroids, but resolved during 48 h after the introduction of dapsone. No adverse effects of treatment were observed. CONCLUSION: Dapsone, a sulphone with an anti-inflammatory activity, showed remarkable therapeutic efficacy against rash and gastrointestinal symptoms in children with HSP. Its administration should be considered particularly in persistent cutaneous form of HSP.

• MeSH
• Skin Diseases, Bacterial drug therapy   MeSH
• Chlamydophila pneumoniae MeSH
• Dapsone therapeutic use   MeSH
• Child MeSH
• Gastrointestinal Diseases drug therapy   MeSH
• IgA Vasculitis complications   MeSH
• Chlamydophila Infections complications   MeSH
• Humans MeSH
• Adolescent MeSH
• Mycoplasma pneumoniae MeSH
• Pneumonia, Mycoplasma complications   MeSH
• Child, Preschool MeSH
• Treatment Outcome MeSH
• Check Tag
• Child MeSH
• Humans MeSH
• Adolescent MeSH
• Child, Preschool MeSH
• Publication type
• Journal Article MeSH
• Case Reports MeSH