Complete remission of nephrotic syndrome in a woman with renal amyloidosis due to Familial Mediterranean Fever

Anti-interleukin 1 agents are used successfully in colchicine-resistant or intolerant Familial Mediterranean Fever (FMF) patients. Sixty-five patients with FMF who received canakinumab treatment for at least 6 months due to colchicine resistance or intolerance between 2016 and 2020 in our department were retrospectively analyzed. Canakinumab treatment was given subcutaneously every 4 weeks. After completing monthly canakinumab therapy over 12 months, in patients with complete remission, the dosing interval was extended to every 1.5 months for 6 months, then every 2 months for 6 months, and finally every 3 months for a year. In patients without disease activation, canakinumab treatment was discontinued at the end of 3 years and followed up with colchicine treatment. Patients who had a flare switched to the previous dosing interval. In patients with renal amyloidosis, monthly canakinumab treatment was continued without extending the dose intervals. The mean duration of canakinumab use in our patients was 31.4 ± 10.57 months (6–52 months). The mean age at onset of symptoms was 4.65 ± 3.84 (range, 1–18) years, and the mean age at diagnosis was 5.59 ± 3.9 (range, 4–19) years. Complete remission was achieved in 57 (87.6%) and partial remission in seven (10.7%) patients. One patient was unresponsive to treatment. Canakinumab treatment was discontinued in three patients with complete remission and one patient with drug resistance. Erythrocyte sedimentation rate (ESR) (51.85 ± 15.7 vs. 27.80 ± 13.73 mm/h) and C-reactive protein (CRP) [26 (3-73) vs. 5 (1–48) mg/L] values were compared before and after canakinumab treatment in attack-free periods, a significant decrease was found after canakinumab treatment (p < 0.001, p < 0.001, respectively). Bodyweight Z-scores (respectively −0.80 ± 0.86 vs. −0.49 ± 0.92) were compared, similarly, a statistically significant increase after canakinumab treatment (p < 0.001), but no significant increase in height Z scores (−1.00 ± 0.88 vs. −0.96 ± 0.94) (p = 0.445) was detected. Four patients had FMF-related renal amyloidosis. The decrease in proteinuria with canakinumab treatment was not statistically significant (p = 0.068). Cervical lymphadenitis developed in one and local reactions in two patients. No severe adverse effects requiring discontinuation of canakinumab treatment were observed. Our study showed that canakinumab treatment was highly effective, well-tolerated in pediatric FMF patients, and controlled extension of the canakinumab dose interval was safe.

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Regression of nephrotic syndrome due to amyloidosis secondary to familial Mediterranean fever following colchicine treatment

Nephrology Dialysis Transplantation ◽

10.1093/ndt/15.2.281 ◽

2000 ◽

Vol 15 (2) ◽

pp. 281-282 ◽

Cited By ~ 10

Author(s):

B Şimşek ◽

İ İşlek ◽

T Şimşek ◽

Ş Küçüködük ◽

K Cengiz

Keyword(s):

Nephrotic Syndrome ◽

Familial Mediterranean Fever ◽

Colchicine Treatment ◽

Mediterranean Fever

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Living kidney transplantation between brothers with unrecognized renal amyloidosis as the first manifestation of familial Mediterranean fever: a case report

BMC Medical Genetics ◽

10.1186/s12881-017-0457-9 ◽

2017 ◽

Vol 18 (1) ◽

Cited By ~ 2

Author(s):

Ramón Peces ◽

Sara Afonso ◽

Carlos Peces ◽

Julián Nevado ◽

Rafael Selgas

Keyword(s):

Kidney Transplantation ◽

Case Report ◽

Familial Mediterranean Fever ◽

Renal Amyloidosis ◽

Mediterranean Fever

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Renal amyloidosis in familial Mediterranean fever

Kidney International ◽

10.1111/j.1523-1755.2004.00485.x ◽

2004 ◽

Vol 65 (3) ◽

pp. 1118-1127 ◽

Cited By ~ 26

Author(s):

Seza Ozen

Keyword(s):

Familial Mediterranean Fever ◽

Renal Amyloidosis ◽

Mediterranean Fever

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A Proposed Histopathologic Classification, Scoring, and Grading System for Renal Amyloidosis: Standardization of Renal Amyloid Biopsy Report

Archives of Pathology & Laboratory Medicine ◽

10.5858/134.4.532 ◽

2010 ◽

Vol 134 (4) ◽

pp. 532-544 ◽

Cited By ~ 10

Author(s):

Sait Şen ◽

Banu Sarsık

Keyword(s):

Familial Mediterranean Fever ◽

Prognostic Score ◽

Renal Amyloidosis ◽

Grading System ◽

Al Amyloidosis ◽

Pathology Report ◽

Aa Amyloidosis ◽

Microscopic Appearance ◽

Biopsy Report ◽

Mediterranean Fever

Abstract Context.—A disease associated with amyloid deposits, called amyloidosis, is associated with characteristic electron microscopic appearance, typical x-ray pattern, and specific staining. Renal involvement mainly occurs in AA amyloidosis and AL amyloidosis and usually progresses to renal failure. Objective.—The renal histopathologic changes with amyloidosis comprise a spectrum. Clear relationships between the extent of amyloid deposition and the severity of clinical manifestations have not been demonstrated. Whether there is a lack of clinicopathologic correlation is not clear, but studies have revealed the need for standardization of the renal amyloid biopsy report. With these objectives in mind, we proposed a histopathologic classification, scoring, and grading system. Renal amyloidosis was divided into 6 classes, similar to the classification of systemic lupus erythematosus. Amyloid depositions and other histopathologic lesions were scored. The sum of these scores was termed the renal amyloid prognostic score and was divided into 3 grades. Data Sources.—AA amyloidosis was detected in 90% of cases, mostly related to familial Mediterranean fever. Positive correlations between class I and grade I, class VI and grade III, and class III and grade II were observed. Also, a positive correlation was identified between severity of glomerular amyloid depositions, interstitial fibrosis, and inflammation. Because of the inadequacy of the patients' records and outcomes, different therapy regimes, and etiologies, clinical validation of this study has not been completed. Conclusions.—Standardization of the renal amyloid pathology report might be critical for patients' medication and comparison of outcome and therapeutic trials between different clinics. Because of our AA to AL amyloidosis ratio and the predisposition of familial Mediterranean fever–related AA amyloidosis, there is a need for further international collaborative studies.

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