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Concept: Vasculitis

194

There is strong diurnal variation in the symptoms and severity of chronic inflammatory diseases, such as rheumatoid arthritis. In addition, disruption of the circadian clock is an aggravating factor associated with a range of human inflammatory diseases. To investigate mechanistic links between the biological clock and pathways underlying inflammatory arthritis, mice were administered collagen (or saline as a control) to induce arthritis. The treatment provoked an inflammatory response within the limbs, which showed robust daily variation in paw swelling and inflammatory cytokine expression. Inflammatory markers were significantly repressed during the dark phase. Further work demonstrated an active molecular clock within the inflamed limbs and highlighted the resident inflammatory cells, fibroblast-like synoviocytes (FLSs), as a potential source of the rhythmic inflammatory signal. Exposure of mice to constant light disrupted the clock in peripheral tissues, causing loss of the nighttime repression of local inflammation. Finally, the results show that the core clock proteins CRYPTOCHROMES 1 and 2 repressed inflammation within the FLSs, and provide novel evidence that a CRYPTOCHROME activator has anti-inflammatory properties in human cells. We conclude that under chronic inflammatory conditions, the clock actively represses inflammatory pathways during the dark phase. This interaction has exciting potential as a therapeutic avenue for treatment of inflammatory disease.-Hand, L. E., Hopwood, T. W., Dickson, S. H., Walker, A. L., Loudon, A. S. I., Ray D. W., Bechtold, D. A., Gibbs, J. E. The circadian clock regulates inflammatory arthritis.

Concepts: Inflammation, Interleukin 1, Asthma, Rheumatoid arthritis, Vasculitis, Anti-inflammatory, Circadian rhythm, C-reactive protein

167

Kawasaki disease (KD) is a systemic vasculitis and affects many organ systems. It often presents sterile pyuria, microscopic hematuria, and proteinuria due to renal involvement. The aims of this study were to define clinical characteristics of acute KD patients with pyuria and to analyze meaning of pyuria in KD.

Concepts: Nephrology, Vasculitis, Nephrotic syndrome, Arthralgia, Glomerulonephritis, Kawasaki disease, Urinalysis, IgA nephropathy

153

A patient with a known biopsy of polyarteritis nodosa diagnosis presented with cyclic fevers, acute kidney injury, and progression of rash from macular to pustular, worsening despite being on antibiotics, without evidence of infection on multiple cultures. The patient had a pathological diagnosis from a skin biopsy of acute generalized exanthematous pustulosis syndrome, with a total resolution of rash, fevers, and acute kidney injury on treatment with pulse steroids.

Concepts: Inflammation, Biopsy, Pathology, Rheumatology, Vasculitis, Acute kidney injury, Polyarteritis nodosa, Diseases involving the fasciae

153

Kawasaki disease (KD) is the most common pediatric vasculitis. The study aimed to identify the risk factors of intravenous immunoglobulin (IVIG) unresponsiveness from the initial clinical parameters of the Taiwanese KD patients.

Concepts: Immune system, Antibody, Vasculitis, Wegener's granulomatosis, Intravenous immunoglobulin, X-linked agammaglobulinemia, Kawasaki disease

31

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a severe condition encompassing two major syndromes: granulomatosis with polyangiitis (formerly known as Wegener’s granulomatosis) and microscopic polyangiitis. Its cause is unknown, and there is debate about whether it is a single disease entity and what role ANCA plays in its pathogenesis. We investigated its genetic basis.

Concepts: Vasculitis, Wegener's granulomatosis, Autoimmune diseases, Arthritis, Granuloma, Anti-neutrophil cytoplasmic antibody, Proteinase 3, Vascular-related cutaneous conditions

29

More than 14 years of clinical practice in rheumatology led the author to discover the prognostic role of anti-citrullinated protein antibody (ACPA) as well as the erosions found by MRI, in detecting the RA patients resulting in establishing a new set of criteria by revising the 1987 ACR classification-Iran Criteria for Rheumatoid Arthritis. Medical records of 243 patients at the outpatient Rheumatology Clinic of the author (private sector) were reviewed for the data on the criteria of the 1987 ACR, 2010 ACR/European League against Rheumatism (EULAR), and Iran Criteria for RA. In addition to modifying the 1987 ACR classification, Iran Criteria for RA adds some additional information to the ACR criteria (including ACPA and bony erosions detected by MRI), and any patient who satisfies 6 out of 12 points is considered as a definite RA patient. Sensitivity of the three classifications was calculated considering the clinical diagnosis by a single rheumatologist as the gold standard. A total of 63 male and 180 female patients with a mean follow-up duration of 28.24 ± 50.19 months were considered. Mean age at diagnosis and mean disease duration were 49.16 ± 15.38 years and 7.04 ± 6.87 months, respectively. The sensitivity for Iran Criteria for RA, 1987 ACR classification, and 2010 ACR/EULAR criteria were calculated as 98.4, 59.7, and 66.3%, respectively. Comparing Iran Criteria for RA with ACR and ACR/EULAR criteria, it was concluded that our newly introduced criteria is a more sensitive instrument in determining RA patients in the early stages of the disease.

Concepts: Medical terms, Rheumatoid arthritis, Rheumatology, Vasculitis, Gout, Arthritis, Rheumatism, European League Against Rheumatism

28

To compare the diagnostic accuracy of the 2010 American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) and 1987 ACR criteria for rheumatoid arthritis (RA) in a cohort of patients with recent-onset arthritis followed-up for 10 years.

Concepts: Medical terms, Rheumatoid arthritis, Rheumatology, Vasculitis, Arthritis, Rheumatism, European League Against Rheumatism

28

Vasculitis is a destructive inflammatory process affecting blood vessels. Pulmonary vasculitis may develop secondary to other conditions or constitute a primary idiopathic disorder. Thoracic involvement is most common in primary idiopathic large-vessel vasculitides (Takayasu arteritis, giant cell arteritis, Behçet disease) and primary antineutrophil cytoplasmic autoantibody-associated small-vessel vasculitides (Wegener granulomatosis, microscopic polyangiitis, Churg-Strauss syndrome). Primary pulmonary vasculitides are rare, and their signs and symptoms are nonspecific, overlapping with those of infections, connective tissue diseases, and malignancies. The radiologic findings in primary pulmonary vasculitis vary widely and can include vessel wall thickening, nodular or cavitary lesions, ground-glass opacities, and consolidations, among others. Diffuse alveolar hemorrhage usually results from primary small-vessel vasculitis in the lungs. To diagnose vasculitis, medical teams must recognize characteristic combinations of clinical, radiologic, laboratory, and histopathologic features.

Concepts: Inflammation, Blood, Blood vessel, Rheumatology, Vasculitis, Wegener's granulomatosis, Giant cell arteritis, Vascular-related cutaneous conditions

28

INTRODUCTION: Wegener’s granulomatosis presenting as diffuse alveolar hemorrhage is uncommon. However, the recognition of multisystem disease involving joints, kidney, eye and lung is critical for diagnosing Wegener’s vasculitis. This is not the first report of this kind in the literature. CASE PRESENTATION: A 51-year-old Croatian woman presented to our Emergency Department with a history of progressively worsening productive cough and shortness of breath, epistaxis and two episodes of hemoptysis. She developed respiratory failure due to diffuse alveolar hemorrhage, which was successfully treated with high-dose steroids, cyclophosphamide and plasmapheresis. Her clinical course was complicated with methicillin-resistant Staphyloccocus aureus pneumonia, which has been associated with Wegener’s granulomatosis flares. CONCLUSION: The recognition of multisystem disease is critical for diagnosing Wegener’s vasculitis. Diffuse alveolar hemorrhage can be a fulminant manifestation of Wegener’s granulomatosis, in which case immediate and aggressive treatment with pulse steroids, high-dose cyclophosphamide and plasma exchange can be life-saving.

Concepts: Pulmonology, Pneumonia, Vasculitis, Wegener's granulomatosis, Arthritis, Granuloma, Plasmapheresis, Hemoptysis

27

Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis that typically occurs without detectable antineutrophil cytoplasmic antibody. It leads to aneurysm formation by affecting muscular arteries, usually those of medium size but also occasionally those of small size. Kidney involvement is common, leading to reduced glomerular filtration rate, hypertension, rupture of renal arterial aneurysms causing perinephric hematomas, and renal infarctions in those with severe vasculitis. Similar to PAN, microscopic polyangiitis (MPA) leads to aneurysm formation; however, MPA usually is associated with antineutrophil cytoplasmic antibody, and glomerulonephritis is a more common feature of MPA. Although kidney biopsy may show classic vascular changes in both PAN and MPA, this procedure is not without risk of significant bleeding due to aneurysm rupture. We present 2 cases of renal aneurysms that were diagnosed as MPA using computed tomography angiography (CTA), allowing implementation of appropriate immunosuppressive therapy. Follow-up CTA after treatment showed resolution of all previously observed abnormalities. CTA is a useful alternative to kidney biopsy in establishing both the extent of disease in renal aneurysms and allowing for tracking of disease progression and response to therapy.

Concepts: Renal failure, Kidney, Nephrology, Atherosclerosis, Blood pressure, Vasculitis, Polyarteritis nodosa, Microscopic polyangiitis