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Sicca-like syndrome in type v hyperlipoproteinemia.

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114
SICCA-LIKE SYNDROME IN TYPE V HYPERLIPOPROTEINEMIA
JAMES L. REINERTSEN, ERNST J. SCHAEFER, H. BRYAN BREWER, and HARALAMPOS M. MOUTSOPOULOS
Patients with type-I1 and type-IV hyperlipoproteinemia may also have four rheumatic problems. First, younger patients with type-I1 disease develop migratory polyarthralgias that resemble
rheumatic fever (1). Second, a variety of miscellaneous
musculoskeletal complaints occur in patients with typeI1 and type-IV hyperlipoproteinemia; such symptoms
include arthralgias with morning gel phenomenon, “palindromic rheumatism,” and tenosynovitis (24). Third,
hyperuricemia and gout often are associated with hypertriglyceridemia (type-IV) (5). Finally, Goldman and
Julian (6) and Kaltreider and Tala1 (7) have described
parotid enlargement similar to that of Sjogren’s syndrome in 13 patients, 12 whom had type-I1 or type-IV
hyperlipoproteinemia. The remaining patient had typeV hyperlipoproteinemia.
The Sjogren-like syndrome of these hyperlipoproteinemias is characterized primarily by parotid
enlargement, without prominent ocular or oral sicca
symptoms, and has been attributed to fatty infiltration
of the parotids (6,7). The incidence of such parotid enlargement and the frequency of associated sicca symptoms in the hyperlipoproteinemias is not known. Furthermore, non-parotid rheumatic symptoms have not
been described in type-V hyperlipoproteinemia. Ac-
James L. Reinertsen, MD: Arthritis and Rheumatism
Branch, National Institute of Arthritis, Metabolism and Digestive
Diseases; Ernst J. Schaefer, MD: Molecular Disease Branch, National
Heart, Lung, and Blood Institute; H. Bryan Brewer, MD: Molecular
Disease Branch, National Heart, Lung, and Blood Institute; Haralampos M. Moutsopoulos, MD: Clinical Immunology Section, National Institute of Dental Research, National Institutes of Health, Bethesda, Maryland 20205.
Address reprint requests to Dr. James L. Reinertsen, St.
Louis Park Medical Center, 5000 West 39th Street, Minneapolis, Minnesota 55416.
Submitted May 7, 1979; accepted in revised form August 29,
1979.
Arthritis and Rheumatism, Vol. 23, No. 1 (January 1980)
cordingly, we have surveyed 25 patients with type-V hyperlipoproteinemia who were examined at the National
Institutes of Health (NIH) for a history of arthritis or
sicca symptoms or both. Four of the 25 patients had significant sicca symptoms and were studied further. Findings in these patients form the basis of this report.
Patients and methods. The diagnosis of type-V
hyperlipoproteinemia was confirmed in all patients by
the following methods. Blood was obtained in 0.1%
EDTA after an overnight fast (12-14 hours), and the
plasma was separated at 4°C in a refrigerated centrifuge. All subjects were sampled while on an ad libitum
diet and were not receiving medication known to affect
plasma lipoproteins. Plasma cholesterol and triglyceride
levels were measured with an Auto Analyzer I1 (8).
High-density lipoprotein (HDL) cholesterol was measured after heparin-manganese precipitation of plasma
or the 1.006 gm/ml infranate (9). Plasma was ultracentrifuged at its own density (1.006 gm/ml) for 18
hours at 39,000 rpm (4°C) in a Beckman 40.3 rotor
(Beckman Instruments, Fullerton, California), and the
very low-density lipoproteins (VLDL) were separated
from the other plasma lipoproteins by tube slicing (10).
The cholesterol content in the 1.006 gm/ml infranatant
fraction was determined by these methods, and VLDL
and low-density lipoprotein (LDL) were calculated (9).
Plasma and the 1.006 gm/ml supernate and infranate
also were subjected to paper electrophoresis for lipoprotein phenotyping (9). Results of these studies are shown
in Table 1. ’
The four sicca patients also underwent other laboratory studies, including complete blood count,
erythrocyte sedimentation rate, urinalysis, and measurements of uric acid, blood urea nitrogen, triiodothyronine (T3), thyroxine (T4), thyroid stimulating hormone (TSH), calcium, phosphorus, rheumatoid factor,
and antinuclear antibody.
BRIEF REPORTS
115
Table 1. Plasma lipid, lipoprotein, and cholesterol levels in 4 patients with type-V hyperlipoproteinemia
Cholesterol levels+
Cholesterol
Triglyceride
VLDL
LDL
HDL
564*
636
804
265
2529
3339
3885
1872
477
557
74 1
180
67
66
53
71
20
13
10
14
87 + 43
16+ 11
123 + 35
50 & 14
Case 1
Case 2
Case 3
Case 4
Normalt
(N = 1088)
Cholesterol*
189 f 40
* For each case these values represent the mean of 3 determinations.
t + 1 standard deviation.
*
mg %.
All 25 type-V patients were questioned for ocular
and oral features of sicca (Sjogren’s) syndrome. Special
studies were done only in those with sicca symptoms.
The ophthalmologic assessment included Schirmer’s
test (1 1) and slit-lamp examination after rose bengal
staining (12). Parotid gland function was evaluated by
salivary flow rate and salivary gland scintigraphy (13).
Labial biopsy was performed as previously described
(14). The outcomes in the four patients are shown in
Table 2.
Case report. A 5 1-year-old white woman was
noted to have marked hypertriglyceridemia and hypercholesterolemia in the course of evaluation for abdominal pain. Type-V hyperlipoproteinemia and chronic
pancreatitis with pseudocyst were found. She had prominent symptoms of morning stiffness in the small joints
of her hands (including distal interphalangeal joints),
shoulders, and feet. Moreover, she complained of burning, gritty eyes and a constantly dry mouth, which required her to keep a glass of water nearby at all times.
’
Examination revealed mild symmetrical parotid
enlargement. The distal interphalangeal (DIP) and
proximal interphalangeal (PIP) joints had typical Heberden’s and Bouchard’s nodes. Tender, nodular thickening of both palmar and plantar fasciae was present.
The remainder of the musculoskeletal examination was
normal. Hand x-rays showed osteoarthritic changes of
DIP and PIP joints. Biopsy of a tender plantar nodule
revealed fibrous thickening of the plantar aponeurosis.
Results of rheumatologic and sicca studies are
shown in Table 2. Pertinent findings include an elevated
ESR, abnormal Schirmer’s test, punctate corneal erosions, class 111 salivary scintiscan, and normal salivary
flow rate, and labial salivary gland biopsy (Figure 1).
A summary of the clinical and laboratory features of cases 1-4 are presented in Table 2.
Discussion. We surveyed 25 patients with type-V
hyperlipoproteinemia for symptoms of sicca syndrome
and identified 4 women with prominent ocular or oral
sicca symptoms. Although this sort of survey cannot de-
Table 2. Features of rheumatic and sicca syndrome in 4 patients with type-V hyperlipoproteinemia
Age at
Case
Diagnosis
Onset of
symptoms
Musculoskeletal
syndrome*
Salivary
flow
99Tc
salivary
scan
Labial
salivary
biopsy
Sed
rate
RF
uric
ANA Acid
I
51
51
OA
Oral,
ocular
4mm
Normal
Abnormal
class 111
Normal
54
Neg
Neg
4.0
1 mm
Punctate
erosions
of
cornea
Normal
2
45
47
Gout
3
43
45
OA
Oral,
ocular
Ocular
Normal
Normal
61
Neg
Neg
8.6
5.5 mm
Normal
Decreased
Normal
87
Neg
Neg
10.3
1Omm
Normal
Decreased
Abnormal,
class 111
Abnormal,
class 111
Abnormal,
class 111
4
50
48
RA
Oral
Normal
91
Pos
Neg
6.4
Sicca Schirmer’s Slit
symptoms test
lamp
*OA = inflammatory osteoarthritis; RA = rheumatoid arthritis.
BRIEF REPORTS
116
-~~-
~~
~~~~
Figure 1. Lip biopsy from patient with type-V lipoproteinemia.The minor salivary gland is normal (X 130).
Figure 2. Lip biopsy from a patient with Sjogren’ssyndrome. Focal lymphocytic infiltrate of the minor salivary
gland is evident, with acinar destruction (X 80).
BRIEF REPORTS
fine a true incidence, it does suggest that xerostomia and
xerophthalmia are not rare in this lipid disorder.
Ocular and oral dryness can result from a number of different pathologic processes (15). One such
cause, primary Sjogren’s syndrome, is characterized by
lacrimal and salivary gland infiltration by lymphocytes
and plasma cells, as well as a wide array of clinical and
serologic autoimmune phenomena (16). It is important
to distinguish true Sjogren’s syndrome (Figure 2) from
other causes of decreased exocrine secretion, especially
in patients with associated musculoskeletal symptoms.
While superficially similar to true Sjogren’s syndrome, the sicca-like syndrome in the 4 type-V lipoproteinemia patients described here is clearly quite different. Although the presence of an elevated sedimentation
rate, arthritis, sicca symptoms, and low salivary flow
would make the diagnosis of sicca syndrome quite possible in these patients, the finding of normal salivary
gland biopsies makes the diagnosis of true Sjogren’s
syndrome untenable, even in the presence of a positive
rheumatoid factor as in patient 4.
Several features of the sicca syndrome seen in
type-V hyperlipoproteinemia are noteworthy.
Parotid gland enlargement is mild or absent in
117
type-V patients, whereas it is a prominent aspect of
type-IV sicca syndrome (6). Furthermore, fatty infiltration of the salivary glands, seen in type-IV patients
(Figure 3), is not observed in biopsies from patients
with type-V hyperlipidemia.
Goldman and Julian (6) postulate that exocrine
dysfunction in type-IV disease is caused by fatty infiltration of glandular tissue. We cannot extend this theory to type-V patients, since the normal biopsies of
these patients do not explain the marked symptoms of
xerostomia and distinctly abnormal salivary scans. Even
electron microscopy of type-V patients failed to show
any abnormal lipid accumulation or other abnormalities.
The presence of recurrent abdominal pain in all
of our patients and the firm diagnosis of chronic pancreatitis in 3 of 4 patients suggest that both pancreatic
and salivary exocrine function is disturbed by similar
mechanisms in type-V hyperlipoproteinemia. Biopsy of
minor salivary glands sheds little light on the nature of
such a process, but the prominent salivary scan abnormalities indicate that focal inflammatory, infiltrative, or
obstructive lesions of the major salivary glands might be
present. Parotid biopsy would perhaps shed some light
Figure 3. Lip biopsy from a patient with type-IV lipoproteinemia showing intense fatty infiltration (X 130).
BRIEF REPORTS
118
on this relationship, but we did not believe that the risks
of this procedure were justified. In any event, the ontogenic and other similarities between the pancreas and
the parotids suggest strongly that their striking coinvolvement in these patients rests on a common pathologic basis.
This study demonstrates that symptoms of ocular
and oral dryness are not uncommon in type-V hyperlipoproteinemia. Arthritic symptoms and elevated sedimentation rates often are present in these patients, making the distinction between true and pseudo-Sjogren’s
syndrome difficult. Biopsy of minor salivary glands
clearly distinguishes between these two types of sicca
syndromes. Therapy with a lipid-sparing diet and eyedrops appeared to ameliorate the sicca syndrome in
these patients with type-V hyperlipoproteinemia.
Acknowledgment. We would like to thank Dawn
M. Cardascia for her excellent secretarial assistance in
the preparation of this manuscript.
5.
6.
7.
8.
9.
10.
11.
12.
13.
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JH: Musculoskeletal disorders associated with type IV hyperlipoproteinemia. Lancet 2449452, 1972
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Holm S: Keratoconjunctivitis sicca and sicca syndrome.
Acta Ophthalmol (suppl) (Kbh) 33:l-230, 1949
Schall GL, Anderson LG, Wolf RO et al: Xerostomia in
Sjogren’s syndrome. Evaluation by sequential salivary
scintigraphy. JAMA 216:2109-2116, 1971
Chisholm DM, Mason DK: Labial salivary gland biopsy
in Sjogren’s syndrome. J Clin Pathol 21:656-660, 1968
Talal N: Sjogren’s syndrome and connective tissue disease
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Conditions. Eighth edition. Edited by JL Hollander, DJ
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Dwight J. lngle Memorial Award
Perspectives in Biology and Medicine announces the Dwight J. lngle Memorial Award for authors
under 35 for the best English language essay. For details contact Richard L. Landau, MD, Editor,
Perspectives in Biology and Medicine, Culver 403, 1025 E. 57th Street, Chicago, Illinois 60637.
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