Tofacitinib for the treatment for colchicine-resistant familial Mediterranean fever: case-based review

AbstractFamilial Mediterranean fever is characterized by self-limited attacks of serositis and arthritis. However, substantial number of patients suffer from chronic complications of this disease, primarily involving musculoskeletal system. Treatment for these complications is challenging due to limited evidence. Interleukin-1 (IL-1) antagonists, tocilizumab and anti-tumor necrosis factor (anti-TNF) agents are off-label treatment options for the management of chronic manifestations of FMF, such as secondary (AA) amyloidosis, chronic arthritis and sacroiliitis. This paper presents a case series of four FMF patients who are refractory to IL-1 antagonists, anti-TNF agents and tocilizumab, who responded well to tofacitinib. The authors also conducted a comprehensive literature search for studies investigating tofacitinib use in FMF patients. Although still limited, current data suggest that tofacitinib could be a useful treatment option for FMF patients with associated inflammatory comorbid conditions and chronic manifestations of disease.
Source: Rheumatology International - Category: Rheumatology Source Type: research

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AbstractFamilial Mediterranean Fever, a monogenic autoinflammatory disease secondary to MEFV gene mutations in the chromosome 16p13, is characterized by recurrent self-limiting attacks of fever, arthritis, aphthous changes in lips and/or oral mucosa, erythema, serositis. It is caused by dysregulation of the inflammasome, a complex intracellular multiprotein structure, commanding the overproduction of interleukin 1. Familial Mediterranean Fever can be associated with other multifactorial autoinflammatory diseases, as vasculitis and Beh çet disease.Symptoms frequently start before 20  years of age and are chara...
Source: Italian Journal of Pediatrics - Category: Pediatrics Source Type: research
AbstractAmyloidosis secondary to juvenile idiopathic arthritis is a known complication of poorly controlled systemic juvenile idiopathic arthritis (SJIA), occurring in 1 –2% of the patients. The IL-6 inhibitor tocilizumab is effective in controlling systemic signs and symptoms of sJIA and may be of therapeutic benefit in secondary amyloidosis. Herein, we report the clinical timeline of a 10-year boy with sJIA and secondary amyloidosis, who showed a sustained impro vement of systemic symptoms and a reduction in proteinuria with tocilizumab. Compared to the data on adult patients affected with the secondary amyloidosis...
Source: Rheumatology International - Category: Rheumatology Source Type: research
Conclusion: A routine ophthalmic follow-up is recommended for all patients with established or suspected amyloidosis, independent of the biochemical type of the amyloid. Close collaboration between the ophthalmologist and the internist will facilitate a more precise diagnosis of ocular involvement in amyloidosis and allow the multidisciplinary management of these patients.Abbreviations: CD: corneal dystrophy; CLA: corneal lattice amyloidosis; CNS: central nervous system; CT: computed tomography; FAP: familial amyloidotic polyneuropathy; GDLCD: gelatinous drop-like corneal dystrophy; GLN: gelsolin; LCD: lattice corneal dyst...
Source: Seminars in Ophthalmology - Category: Opthalmology Tags: Semin Ophthalmol Source Type: research
We report here a case of nodular pulmonary amyloidosis that evolved over a 10-year period. A 61 year-old woman was referred for a suspicion of pulmonary amyloidosis. She has been diagnosed at the age of 30 with rheumatoid arthritis (RA) that required for years, hydroxychloroquine.
Source: Clinical Lymphoma, Myeloma and Leukemia - Category: Hematology Authors: Source Type: research
CONCLUSION: Canakinumab may be an effective treatment option for pediatric FMF patients with colchicine resistance, renal amyloidosis, and chronic oligoarthritis. Further studies are needed to clarify the efficacy of canakinumab in patients with a second disease, RF-positive polyarticular juvenile idiopathic arthritis. PMID: 31463794 [PubMed - as supplied by publisher]
Source: Paediatric Drugs - Category: Pediatrics Authors: Tags: Paediatr Drugs Source Type: research
ConclusionCanakinumab may be an effective treatment option for pediatric FMF patients with colchicine resistance, renal amyloidosis, and chronic oligoarthritis. Further studies are needed to clarify the efficacy of canakinumab in patients with a second disease, RF-positive polyarticular juvenile idiopathic arthritis.
Source: Pediatric Drugs - Category: Pediatrics Source Type: research
CONCLUSION: Amyloid light-chain amyloidosis arthropathy is probably underdiagnosed. A review of amyloid arthropathy associated with multiple myeloma found that 33% of patients had been misdiagnosed with rheumatoid arthritis. PMID: 31444022 [PubMed - as supplied by publisher]
Source: Revue de Medecine Interne - Category: Internal Medicine Tags: Rev Med Interne Source Type: research
Abstract Immunoglobulin light chains are called free when they are not linked with heavy chains to form a whole immunoglobulin. Quantification of free light chains is a part of the French national authority for health guidelines for diagnostic and follow-up of light chain, oligo or non-secretory myeloma and AL amyloidosis. Most recently, the World health organisation had included free light chains quantification in prognostic criteria for monoclonal gammopathy of undetermined significance. However the literature bring to light some other potential indications of this analysis in the exploration of monoclonal gammo...
Source: Annales de Biologie Clinique - Category: Biochemistry Authors: Tags: Ann Biol Clin (Paris) Source Type: research
Abstract Rheumatoid arthritis (RA) is accompanied by a variety of nephropathies. It is often difficult to distinguish between disease-associated and drug-associated renal diseases. Three hundred and seventy-six RA patients with renal involvement were included in our study; they were subjected to full history and clinical examination, kidney function, 24-h urinary protein, and kidney biopsy. All our patients were on methotrexate, low dose steroids, and nonsteroidal anti-inflammatory drugs, in addition to the previous medications. About 79.3%, 20.7%, 6.9%, and 5.9% of our patients were on leflunomide, hydroxychloroq...
Source: Saudi Journal of Kidney Diseases and Transplantation - Category: Urology & Nephrology Authors: Tags: Saudi J Kidney Dis Transpl Source Type: research
PMID: 31244353 [PubMed - as supplied by publisher]
Source: Scandinavian Journal of Rheumatology - Category: Rheumatology Authors: Tags: Scand J Rheumatol Source Type: research
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