Case Report

“Amyloid Storm” As a Rare Etiology of Acute Kidney Injury: Case Series and Review of Literature

by Ege Sinan Torun1*, Çağlar Çakır2, Orkide Kutlu3, Gülay Kadıoğlu4

1University of Health Sciences, Prof. Dr. Cemil Taşçıoğlu City Hospital, Department of Internal Medicine, Division of Rheumatology, Istanbul, Turkey

2University of Health Sciences, Prof. Dr. Cemil Taşçıoğlu City Hospital, Department of Pathology, Istanbul, Turkey

3University of Health Sciences, Prof. Dr. Cemil Taşçıoğlu City Hospital, Department of Internal Medicine, Istanbul, Turkey

4University of Health Sciences, Prof. Dr. Cemil Taşçıoğlu City Hospital, Department of Internal Medicine, Division of Nephrology, Istanbul, Turkey

*Corresponding author: Ege Sinan Torun, University of Health Sciences, Prof. Dr. Cemil Taşçıoğlu City Hospital, Department of Internal Medicine, Division of Rheumatology, Istanbul, Turkey

Received Date: 17 September 2024

Accepted Date: 20 September 2024

Published Date: 23 September 2024

Citation: Torun ES, Cakir C, Kutlu O, Kadioglu G (2024) “Amyloid Storm” As a Rare Etiology of Acute Kidney Injury: Case Series and Review of Literature. Ann Case Report. 9: 1986. https://doi.org/10.29011/2574-7754.101986

Abstract

Keywords: Amyloidosis, Ankylosing spondylitis, Auto inflammatory diseases.

Introduction

“Amyloid storm” is a recently defined hyper inflammatory condition that causes acute kidney injury. Criteria of “amyloid storm are: Increase of creatinine and proteinuria levels at least two levels and increase of CRP values to at least ten times compared to highest normal level in less than two weeks [1]. Even though it was first reported in patients with AA amyloidosis secondary to Familial Mediterranean Fever (FMF), it has also been described in patients with clinical conditions other than FMF [2]. We aimed to describe the clinical features of our patients who presented with amyloid storm.

Cases

The clinical features of our 3 amyloid storm patients are summarized in Table 1.

Patient 1

Patient 2

Patient 3

Age

33

47

63

Sex

Male

Male

Female

Creatinine at presentation

7.03 mg/dl

12.66 mg/dl

7.99 mg/dl

Proteinuria at presentation

12868 mg/day

5568 mg/day

10766 mg/day

CRP at presentation

78.2 mg/l

209 mg/l

206.3 mg/l

Procalcitonin at presentation

0.56 µg/l

1.81 µg/l

0.53 µg/l

Basal creatinine

0.94 mg/dl

1 mg/dl

1.12 mg/dl

Basal proteinuria

2+ (not quantified)

342 mg/day

1+ (not quantified)

Condition that triggered amyloid storm

Pneumonia

Pneumonia

Lymphadenitis in the right inguinal lymph node

Renal biopsy

AA amyloidosis in glomerular mesangium, tubulus basement memebrane, interstitium and vessel wall

AA amyloidosis in glomerular, mesangium, walls of arterioles and arteries

AA amyloidosis in mesangial nodules, arterioles and capillary walls

Other organs with biopsy proven AA amyloidosis

Stomach, duodenum and colon

-

Bone marrow

Pre-existing rheumatic disease

None

Ankylosing spondylitis

None

Treatment for the rheumatic disease

-

Sulfasalazine, NSAIDs

-

MEFV mutation status

Negative

Negative

Negative

Final diagnosis

Idiopathic AA amiloidosis

AA amyloidosis secondary to ankylosing spondylitis

AA amyloidosis secondary tosystemic inflammatory disease (Unspecified granulomatous disease)

Treatment initiated

Colchicine (0.5 mg/day) + Canakinumab (150 mg /month)

Colchicine (0.5 mg/day) + Adalimumab (40 mg/ 2 weeks)

Colchicine (0.5 mg/day)*

Current status (in terms of survival and renal function)

Alive, continues hemodialysis three times a week, preparing for renal transplantation

Alive, continues hemodialysis three times a week, preparing for renal transplantation

Exitus due to septic shock while on hemodialysis three times a week

Final CRP

5.6 mg/l

6.9 mg/l

269.2 mg/l*

*Patient 3 succumbed to septic shock before Anakinra could be initiated

Table 1: Clinical features of the patients with amyloid storm.

Discussion

Amyloidosis constitutes the etiology of chronic kidney disease in 0.83 % of Turkish patients undergoing routine hemodialysis [3]. Recently, clinicians have begun to encounter patients that present with acute kidney injury and are subsequently diagnosed with amyloidosis, which was defined as “amyloid storm” [1].

FMF amyloidosis patients with “amyloid storm” were younger and associated with higher risk for progression to end stage renal disease (ESRD) and mortality [1]. Two of our patients were younger than 50. All of our patients progressed to ESRD. After one-year follow-up, one patient died due to septic shock.

Cases of amyloid storm were also identified in patients without FMF. Bektaş and colleagues reported 9 patients fulfilling the definition of “amyloid storm”. Five patients had FMF. Two patients had ankylosing spondylitis, 1 patient had amyloidosis secondary to non-FMF auto inflammatory disease, 1 patient had idiopathic AA amyloidosis [2]. None of our 3 patients had clinical symptoms of FMF and MEFV mutations of our patients were negative. Patient 2 has ankylosing spondylitis. Patient 1 and Patient 3 did not have a rheumatological diagnosis. Patient 1 did not have any other findings, therefore we considered him to have “idiopathic” AA amyloidosis. Patient 3 did not have a rheumatological diagnosis. However, her past medical history was notable for the lung biopsy performed 6 months ago, which revealed granulomatous inflammation which was negative for tuberculosis. Additionally, during admission, findings suggestive of systemic granulomatous disease (dermopathic lymphadenopathy with histiocytic infiltration in the inguinal lymph node biopsy and reactive plasmacytosis in the bone marrow biopsy) were detected. However, patient died before a definite diagnosis could be reached.

The most common trigger for amyloid storm was infections [1,2]. Similar to the literature all of our patients presented with infections.

Treatment of the amyloid storm is supportive [1]. All of our patients received supportive treatment and colchicine. Patient 1 and patient 2 received biologic agents (canakinumab and adalimumab respectively) and even though we planned to treat patient 3 with anakinra, her clinical condition deteriorated and she died. CRP of the first two patients decreased to close to normal levels after initialisation of biological agents.

In conclusion, we present three more non-FMF patients that have clinical and renal histopathological findings compatible with amyloid storm triggered by infections and all became hemodialysis dependant.

Informed Consent: Informed consent was obtained from the two patients that are still alive and from the family of the third patient who passed away.

Conflict of Interest: None

References

  1. Kukuy OL, Beckerman P, Dinour D, Ben-Zvi I, Livneh A. (2021) Amyloid storm: acute kidney injury and massive proteinuria, rapidly progressing to end-stage kidney disease in AA amyloidosis of familial Mediterranean fever. Rheumatology (Oxford). 60:3235-3242.
  2. Bektas M, Koca N, Oguz E, Sari S, Dagci G, et al. (2024) Characteristics and course of patients with AA amyloidosis: single centre experience with 174 patients from Turkey. Rheumatology (Oxford). 63:319-328.
  3. Nephrology-Dialysis and Transplantation in Turkey. Registry 2022[in Turkish]. Ankara: Turkish Nephrology Association Publications, 2022.

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