Vascular renal AA amyloidosis in adult Still's disease.

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Nephrol Dial Transplant (1997) 12: 1714–1716
Nephrology
Dialysis
Transplantation
Case Report
Vascular renal AA amyloidosis in adult Still’s disease
F. Rivera1, C. M. Gil1, M. T. Gil1, E. Batlle-Gualda2, M. Trigueros3 and J. Olivares1
1Nephrology, 2Rheumatology, and 3Pathology Services, University General Hospital and Department of Internal Medicine,
Alicante, Spain
Key words: adult Still’s disease; amyloidosis178 mmol/l and creatinine clearance 40 ml/min), with-
out arterial hypertension or oedema. Several routine
urinalyses were normal, without proteinuria or any
abnormality in the urinary sediment. On physical
examination, blood pressure was 140/80 mmHg. The
patient presented typical findings of chronic poly-
arthritis: pain, limitation of movement and structural
Introduction
Renal secondary amyloidosis complicates the evolution
of several chronic rheumatic diseases,especially rheum-
atoid arthritis and ankylosing spondylitis [1,2]. The
association of amyloid deposits and Still’s disease is
rather unusual [3–5] and should be considered a
serious complication [6]. Amyloid may involve all
structures of the kidney, but the glomeruli are often
predominantly a?ected. However, in occasional cases
the small arterioles are the only structure involved.
Such deposits in intrarenal blood vessels have been
described in primary amyloidosis, but rarely in the
secondary forms [7].
We report a case of secondary renal amyloidosis in
adult Still’s disease, with
deposits, manifested by moderate chronic renal failure
without proteinuria.
predominant vascular
Fig. 1. Amorphous acellular deposits of amyloid material that pre-
dominates in the arteriolar wall (H & E×162).
Case report
A 42-year-old man was diagnosed as having adult
Still’s disease 16 years ago when he presented with
high spiking fever,leukocytosis, typical transient macu-
lopapular rash, lymphadenopathy, splenomegaly, and
symmetrical polyarthritis involving hands (metacarpo-
phalangeal, proximal and distal interphalangeal),
wrists, elbows, knees, ankles, and feet (tarsal and
metatarsophalangeal joints). During follow-up he
developed severe typical joint radiographic changes
including carpal and tarsal ankylosis. Infection and
malignancy were excluded. Serum autoantibodies and
rheumatoid factor were persistenly negatives. Owing
to chronic active disease he received aspirin, non-
steroidal anti-inflammatory drugs, prednisone, and
azathioprine, with partial response. In the last year
chronic renal disease was detected (serum creatinine
Fig. 2. Massive infiltration of AA protein in arteriolar wall shown
with immunohistochemical stain (monoclonal antihuman amyloid A
component, combined with immunoperoxidase staining, Dako
DiagnosticsR, ×162). .
Correspondence and o?print requests to: Dr Francisco Rivera,
Facultad de Medicina, Departamento de Medicina, Apartado de
Correos 374, 03080 San Juan, Alicante, Spain.
© 1997 European Renal Association–European Dialysis and Transplant Association

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Vascular renal AA amyloidosis in adult Still’s disease1715
damage in hands, wrist, knees, ankles, and feet, with
mild synovitis in some of them. Laboratory assessment
showed anaemia (serum haemoglobin 10 g/l) and
stable renal function impairment. Serum total proteins,
albumin, and hepatic biochemical parameters were
normal. Abdominal ultrasound examination revealed
small butmorphologically
Percutaneous renal biopsy was performed. H & E
sectionsrevealedglomerular
Interstitial fibrosis with areas of plasma cells and
lymphocytes as well as tubule atrophy and hyaline
casts were noted on histological sections. The most
relevant changes were observed in vessels with amorph-
ous acellular deposits in the arteriolar wall (Figure 1).
After Congo red staining, reddish pink deposits were
found in vascular walls; furthermore bright green fluo-
rescence was viewed with thioflavine T stain under
ultraviolet light. With immunohistochemical stain
using antiserum against AA amyloid protein combined
with immunoperoxidase, protein AA was present
almost exclusively in the blood vessel walls (Figure 2).
The glomeruli showed minimal immunofluorescence
with AA antibodies. The final diagnosis was secondary
vascular renal amyloidosis due to adult Still’s disease.
producing a progressive ischaemia of the renal paren-
chyma with contracted kidneys [14], as we have
observed in our patient. Moreover, the lack of hyper-
tension seems to be associated with vascular amyl-
oidosis despite the progressive development of renal
failure [15]. Obviously, the definitive diagnosis of
vascular amyloidosis must be based on renal histo-
pathology. In fact, in our patient we initially suspected
other diagnostic possibilities, such as analgesic nephro-
pathy or other renal diseases, because of the absence
of proteinuria. Although the interstitial changes
observed on histological sections can be related to
the chronictreatment with analgesic
inflammatory drugs, the principal structures a?ected
were blood vessels.
The mechanisms of vascular amyloid deposition and
the reasons for the di?erences in intrarenal distribution
of amyloid are not well known. Probably, amyloid
fibrils are formed from the deposited precursors by
action of lysosomal enzymes, released in vascular amyl-
oidosis from the endothelial cells. These enzymes have
the capacity to break down precursors of serum AA
amyloid.
In conclusion, vascular amyloidosis must be sus-
pected in adult Still’s disease with unexplained renal
failure without hypertension or proteinuria. In this
situation, renal biopsy is necessary for the definitive
diagnosis.
normal kidneys.
hyalinechanges.
andanti-
Discussion
Adult Still’s diseaseis a disorder of unknown aetiology,
characterized by typical spiking fever with evanescent
rash and the involvement of various organs. Our
patient was diagnosed as su?ering from adult Still’s
disease based on the classical criteriaselected by several
authors [4,8]. Furthermore he had four major criteria
(fever, arthritis, rash, and leukocytosis) and two minor
criteria (lymphadenopathy,antinuclear antibodies,and
rheumatoid factor negatives) for diagnosis of this
disease [9]. In this patient, we found the association
of renal amyloidosis and adult Still’s disease. In fact,
amyloidosis is an infrequent complication of adult
Still’s disease [5,10]. Pouchot et al. [4] reviewed 62
Still’s disease patients and none of them had developed
amyloidosis. The development of amyloidosis occurs
in patients with long-standing persistent inflammatory
disease [8,11], as we have observed in our patient.
When this complication occurs, amyloid material is
preferentially deposited in the glomeruli and pro-
teinuria and nephrotic syndrome are the most common
initial manifestations [12].
The distribution of amyloid deposits in our patient
was unusual, located almost exclusively in arteriolar
walls. A form of predominant vascular amyloid depos-
ition in the kidney in patients with minimal or no
proteinuria has been reported [13]. However, in sec-
ondary amyloidosis, compared to primary forms, the
amyloid deposition is generally found in the mesang-
ium of the glomeruli and less frequently in intrarenal
blood vessels [7]. Vascular renal amyloidosis is charac-
terized by progressive renal failure without proteinuria
because the glomeruli are relatively preserved. The
vascular amyloid deposits obturate the blood vessels
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Received for publication: 20.1.97
Accepted in revised form: 12.3.97