Journal Information
Vol. 36. Issue. 3.May - June 2016
Pages e1-e52Pages 217-332
Vol. 36. Issue. 3.May - June 2016
Pages e1-e52Pages 217-332
Letter to the Editor
DOI: 10.1016/j.nefroe.2016.06.009
Open Access
Complete remission of nephrotic syndrome in a woman with renal amyloidosis due to Familial Mediterranean Fever
Remisión completa de síndrome nefrótico en mujer con amiloidosis renal por fiebre mediterránea familiar
Visits
...
Manuel Herasa,
Corresponding author
, Ana Saizb, María José Fernández-Reyesa, Álvaro Molinaa, María Astrid Rodrígueza, Ramiro Callejasa
a Servicio de Nefrología, Hospital General de Segovia, Segovia, Spain
b Anatomía Patológica, Hospital Ramón y Cajal, Madrid, Spain
Article information
Full Text
Bibliography
Download PDF
Statistics
Tables (1)
Table 1. Clinical course of laboratory values and treatments used.
Full Text
Dear Editor,

Chronic kidney disease due to AA amyloidosis is one of the main complications of Familial Mediterranean Fever (FMF).1 In 2009, in the journal Nefrología, we presented the case of a 38-year-old woman of Armenian origin with severe nephrotic syndrome due to AA amyloidosis as a form of onset of FMF (with heterozygous M680I and M694V mutations in the MEFV gene). Given the severity of her proteinuria at diagnosis, deterioration in renal function and intolerance to antiproteinuric drugs, we started treatment with colchicine, at doses of 0.5mg/8h/day, and infliximab, at doses of 5mg/kg IV at baseline, at 2 weeks and subsequently every 2 months. In the clinical follow-up performed in the first year of treatment to evaluate her response to this, we reported partial remission: clinical improvement and improvement in renal function, but with persistent nephrotic proteinuria.2

In this report we describe her mid-term clinical course. In the following 6 years, the patient has remained asymptomatic with no new episodes of hydropic decompensation or hospital admissions. Table 1 shows the clinical course of both her laboratory values and the treatment followed. Starting from the second year of treatment, colchicine was sustained continuously at doses of 1mg/day. Given that her proteinuria progressively decreased, infliximab doses were increasingly spaced out, to intervals of 4–6 months, and this treatment was permanently suspended in 2011.

Table 1.

Clinical course of laboratory values and treatments used.

As a result of combination therapy with colchicine and infliximab, in the acute phase of diagnosis of AA amyloidosis due to FMF, clinical improvement and improvement in renal function were achieved, but nephrotic proteinuria persisted. Subsequent follow-up required us to modify our initial conclusions: her clinical improvement was maintained, without any new hydropic decompensation, in the context of resolution of her proteinuria.

Treatment with colchicine is effective in preventing amyloidosis in Armenian patients with FMF.3 In cases of colchicine resistance or intolerance, anti-TNF agents may be effective to treat these patients and manage symptoms associated with FMF.4,5 In our case, given the initial severity of nephrotic syndrome, together with the findings of AA amyloid in the renal parenchyma and the M694V mutation associated with the most serious cases, combination therapy was decided upon, with a partial response in the first year and subsequently a complete response sustained over time. Perhaps this combination treatment, as it prevented the onset of new autoinflammatory episodes associated with FMF, would prevent more amyloid from being deposited in the renal tissue and, furthermore, progressive regression of the amyloid accumulated. In this case, we cannot confirm this, lacking new histology. However, the signs and symptoms may suggest it, as resolution of her proteinuria was achieved. In this regard Kutlugün et al. also reported in the literature 2 cases of complete remission of nephrotic syndrome with colchicine 1.5mg/day, sustained for a period of close to 30 years.6

In conclusion, the main kidney complication (chronic kidney disease) associated with AA amyloidosis due to FMF could be prevented through a resolution of proteinuria, which could be achieved with initial intensive combination therapy (colchicine+anti-TNF agent) and subsequent maintenance with colchicine.

References
[1]
T. Sarkisian, H. Ajrapetian, A. Beglarian, G. Shahsuvarian, A. Egiazarian.
Familial Mediterranean Fever in Armenian population.
Georgian Med News, 156 (2008), pp. 105-111
[2]
M. Heras, R. Sánchez, A. Saiz, M.J. Fernández-Reyes, A. Molina, F. Alvarez-Ude.
Amiloidosis renal en mujer con fiebre mediterránea familiar: respuesta clínica al tratamiento con colchicina e infliximab.
[3]
M.K. Sevoyan, T.F. Sarkisian, A.A. Beglaryan, G.R. Shahsuvaryan, H.K. Armenian.
Prevention of amyloidosis in familial Mediterranean fever with colchicine: a case–control study in Armenia.
Med Princ Pract, 18 (2009), pp. 441-446
[4]
S. Erten, S.F. Erten, A. Altunoglu.
Successful treatment with anti-tumor necrosis factor (anti-TNF)-alpha of proteinuria in a patient with Familial Mediterranean fever (FMF) resistant to colchicines: anti-TNF drugs and FMF.
Rheumatol Int, 32 (2012), pp. 1095-1097
[5]
S. Ozgocmen, O. Akgul.
Anti-TNF agents in Familial Mediterranean fever: report of three cases of review of the literature.
Mod Rheumatol, 21 (2011), pp. 684-690
[6]
A. Kutlugün, T. Yildirim, M. Altindal, M. Arici, U. Yasavul, C. Turgan.
AA type renal amyloidosis secondary to FMF: a long-term follow-up in two patients.
Ren Fail, 32 (2010), pp. 1230-1232

Please cite this article as: Heras M, Saiz A, Fernández-Reyes MJ, Molina A, Astrid Rodríguez M, Callejas R. Remisión completa de síndrome nefrótico en mujer con amiloidosis renal por fiebre mediterránea familiar. Nefrologia. 2016;36:331–332.

Copyright © 2015. Sociedad Española de Nefrología
Idiomas
Nefrología (English Edition)

Subscribe to our newsletter

Article options
Tools
es en

¿Es usted profesional sanitario apto para prescribir o dispensar medicamentos?

Are you a health professional able to prescribe or dispense drugs?

es en
Política de cookies Cookies policy
Utilizamos cookies propias y de terceros para mejorar nuestros servicios y mostrarle publicidad relacionada con sus preferencias mediante el análisis de sus hábitos de navegación. Si continua navegando, consideramos que acepta su uso. Puede cambiar la configuración u obtener más información aquí. To improve our services and products, we use "cookies" (own or third parties authorized) to show advertising related to client preferences through the analyses of navigation customer behavior. Continuing navigation will be considered as acceptance of this use. You can change the settings or obtain more information by clicking here.