| Rank |
Title |
Journal |
Year |
PubWeight™‹?› |
|
1
|
Spectrum of clinical features in Muckle-Wells syndrome and response to anakinra.
|
Arthritis Rheum
|
2004
|
7.07
|
|
2
|
Interleukin-1-receptor antagonist in the Muckle-Wells syndrome.
|
N Engl J Med
|
2003
|
3.86
|
|
3
|
Eprodisate for the treatment of renal disease in AA amyloidosis.
|
N Engl J Med
|
2007
|
3.38
|
|
4
|
Association of mutations in the NALP3/CIAS1/PYPAF1 gene with a broad phenotype including recurrent fever, cold sensitivity, sensorineural deafness, and AA amyloidosis.
|
Arthritis Rheum
|
2002
|
2.58
|
|
5
|
Infusion of pharmaceutical-grade natural human C-reactive protein is not proinflammatory in healthy adult human volunteers.
|
Circ Res
|
2013
|
2.26
|
|
6
|
Cyclophosphamide, bortezomib, and dexamethasone therapy in AL amyloidosis is associated with high clonal response rates and prolonged progression-free survival.
|
Blood
|
2012
|
1.80
|
|
7
|
Safety and efficacy of risk-adapted cyclophosphamide, thalidomide, and dexamethasone in systemic AL amyloidosis.
|
Blood
|
2006
|
1.54
|
|
8
|
Heterogeneity among patients with tumor necrosis factor receptor-associated periodic syndrome phenotypes.
|
Arthritis Rheum
|
2003
|
1.51
|
|
9
|
Familial Mediterranean fever caused by homozygous E148Q mutation complicated by Budd-Chiari syndrome and polyarteritis nodosa.
|
Rheumatology (Oxford)
|
2010
|
1.44
|
|
10
|
Inflammatory bowel disease and systemic AA amyloidosis.
|
Dig Dis Sci
|
2013
|
1.43
|
|
11
|
Efficacy of bortezomib in systemic AL amyloidosis with relapsed/refractory clonal disease.
|
Haematologica
|
2008
|
1.38
|
|
12
|
Successful treatment of familial Mediterranean fever with Anakinra and outcome after renal transplantation.
|
Nephrol Dial Transplant
|
2008
|
1.37
|
|
13
|
An international registry on autoinflammatory diseases: the Eurofever experience.
|
Ann Rheum Dis
|
2012
|
1.36
|
|
14
|
Online registry for mutations in hereditary amyloidosis including nomenclature recommendations.
|
Hum Mutat
|
2014
|
1.23
|
|
15
|
Sequential heart and autologous stem cell transplantation for systemic AL amyloidosis.
|
Blood
|
2005
|
1.22
|
|
16
|
Outcome in renal Al amyloidosis after chemotherapy.
|
J Clin Oncol
|
2011
|
1.21
|
|
17
|
Senile systemic amyloidosis: clinical features at presentation and outcome.
|
J Am Heart Assoc
|
2013
|
1.20
|
|
18
|
AL amyloidosis associated with IgM paraproteinemia: clinical profile and treatment outcome.
|
Blood
|
2008
|
1.20
|
|
19
|
Outcome of autologous stem cell transplantation for AL amyloidosis in the UK.
|
Br J Haematol
|
2006
|
1.13
|
|
20
|
Phenotype, genotype, and sustained response to anakinra in 22 patients with autoinflammatory disease associated with CIAS-1/NALP3 mutations.
|
Arch Dermatol
|
2006
|
1.12
|
|
21
|
Systemic amyloidosis in England: an epidemiological study.
|
Br J Haematol
|
2013
|
1.08
|
|
22
|
Diagnosis, pathogenesis, treatment, and prognosis of hereditary fibrinogen A alpha-chain amyloidosis.
|
J Am Soc Nephrol
|
2008
|
1.07
|
|
23
|
Amyloidogenicity and clinical phenotype associated with five novel mutations in apolipoprotein A-I.
|
Am J Pathol
|
2011
|
1.04
|
|
24
|
Sustained pharmacological depletion of serum amyloid P component in patients with systemic amyloidosis.
|
Br J Haematol
|
2010
|
1.04
|
|
25
|
Translocations of 14q32 and deletions of 13q14 are common chromosomal abnormalities in systemic amyloidosis.
|
Br J Haematol
|
2002
|
1.04
|
|
26
|
The electrocardiographic features associated with cardiac amyloidosis of variant transthyretin isoleucine 122 type in Afro-Caribbean patients.
|
Am Heart J
|
2012
|
1.03
|
|
27
|
Guidelines on the diagnosis and investigation of AL amyloidosis.
|
Br J Haematol
|
2014
|
1.01
|
|
28
|
Sustained remission of symptoms and improved health-related quality of life in patients with cryopyrin-associated periodic syndrome treated with canakinumab: results of a double-blind placebo-controlled randomized withdrawal study.
|
Arthritis Res Ther
|
2011
|
1.00
|
|
29
|
A preliminary score for the assessment of disease activity in hereditary recurrent fevers: results from the AIDAI (Auto-Inflammatory Diseases Activity Index) Consensus Conference.
|
Ann Rheum Dis
|
2010
|
0.96
|
|
30
|
AA amyloidosis complicating the hereditary periodic fever syndromes.
|
Arthritis Rheum
|
2013
|
0.94
|
|
31
|
Neurologic manifestations of the cryopyrin-associated periodic syndrome.
|
Neurology
|
2010
|
0.93
|
|
32
|
Brief Report: whole-exome sequencing revealing somatic NLRP3 mosaicism in a patient with chronic infantile neurologic, cutaneous, articular syndrome.
|
Arthritis Rheumatol
|
2014
|
0.93
|
|
33
|
Additive loss-of-function proteasome subunit mutations in CANDLE/PRAAS patients promote type I IFN production.
|
J Clin Invest
|
2015
|
0.90
|
|
34
|
Guidelines on the management of AL amyloidosis.
|
Br J Haematol
|
2014
|
0.87
|
|
35
|
Cardiac phenotype and clinical outcome of familial amyloid polyneuropathy associated with transthyretin alanine 60 variant.
|
Eur Heart J
|
2011
|
0.87
|
|
36
|
Involvement of X-box binding protein 1 and reactive oxygen species pathways in the pathogenesis of tumour necrosis factor receptor-associated periodic syndrome.
|
Ann Rheum Dis
|
2012
|
0.86
|
|
37
|
The emerging role of interleukin-1β in autoinflammatory diseases.
|
Curr Allergy Asthma Rep
|
2011
|
0.86
|
|
38
|
MEFV mutations affecting pyrin amino acid 577 cause autosomal dominant autoinflammatory disease.
|
Ann Rheum Dis
|
2013
|
0.86
|
|
39
|
Clinical characteristics in subjects with NLRP3 V198M diagnosed at a single UK center and a review of the literature.
|
Arthritis Res Ther
|
2013
|
0.85
|
|
40
|
Cardiac amyloidosis, a monoclonal gammopathy and a potentially misleading mutation.
|
Nat Clin Pract Cardiovasc Med
|
2008
|
0.82
|
|
41
|
Lenalidomide and dexamethasone for systemic AL amyloidosis following prior treatment with thalidomide or bortezomib regimens.
|
Br J Haematol
|
2014
|
0.82
|
|
42
|
Hypercalcemia in a patient with common variable immunodeficiency and renal granulomas.
|
Am J Kidney Dis
|
2005
|
0.80
|
|
43
|
Familial amyloidotic polyneuropathy with severe renal involvement in association with transthyretin Gly47Glu in Dutch, British and American-Finnish families.
|
Amyloid
|
2004
|
0.80
|
|
44
|
Abnormal N-terminal fragment of brain natriuretic peptide in patients with light chain amyloidosis without cardiac involvement at presentation is a risk factor for development of cardiac amyloidosis.
|
Haematologica
|
2011
|
0.79
|
|
45
|
Systemic AA amyloidosis.
|
Subcell Biochem
|
2012
|
0.79
|
|
46
|
Efficacy and safety of canakinumab therapy in paediatric patients with cryopyrin-associated periodic syndrome: a single-centre, real-world experience.
|
Rheumatology (Oxford)
|
2013
|
0.78
|
|
47
|
Clinical characteristics and SAP scintigraphic findings in 10 patients with AGel amyloidosis.
|
Amyloid
|
2014
|
0.78
|
|
48
|
Additive loss-of-function proteasome subunit mutations in CANDLE/PRAAS patients promote type I IFN production.
|
J Clin Invest
|
2016
|
0.77
|
|
49
|
Clinical profile and treatment outcomes of immunoglobulin D associated AL amyloidosis.
|
Br J Haematol
|
2013
|
0.77
|
|
50
|
A prospective study of nutritional status in immunoglobulin light chain amyloidosis.
|
Haematologica
|
2012
|
0.76
|
|
51
|
High-dose melphalan versus melphalan plus dexamethasone for AL amyloidosis.
|
N Engl J Med
|
2008
|
0.76
|
|
52
|
A new era in the treatment of amyloidosis?
|
N Engl J Med
|
2013
|
0.75
|
|
53
|
Hereditary fibrinogen A alpha-chain amyloidosis: clinical phenotype and role of liver transplantation.
|
Blood
|
2010
|
0.75
|
|
54
|
A novel transthyretin variant p.H110D (H90D) as a cause of familial amyloid polyneuropathy in a large Irish kindred.
|
Amyloid
|
2014
|
0.75
|
|
55
|
CMR-Verified Regression of Cardiac AL Amyloid After Chemotherapy.
|
JACC Cardiovasc Imaging
|
2017
|
0.75
|