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Features of systemic lupus erythematosus and sarcoidosis occurring together.

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Chronic diffuse pulmonary fibrosis has previously been reported as a feature of systemic lupus erythematosus (1-5). However, the histologic finding of
granulomas in the lungs of patients with systemic lupus
erythematosus has been rarely reported (6). We report a
patient with clinical and laboratory features of systemic
lupus erythematosus who, on the basis of noncaseating
granulomas found at lung biopsy, was diagnosed as
having sarcoidosis.
Case report. EB, a 62-year-old white woman,
was well until age 25 years when she developed gradual
onset of pain in the shoulders, wrists, metacarpophalangeal (MCP) and proximal interphalangeal (PIP)
joints, knees, ankles, and metatarsophalangeal (MTP)
joints. These joint pains were persistent until she was 30
and then became episodic. At 29 she developed hypothyroidism and has received thyroid replacement since
then. At age 48 years she developed dry eyes and
In 1971 at age 54 years she presented with episodic polyarthritis, dry eyes and mouth, increased hair
loss in the temporal regions, a cough productive of clear
sputum, and progressive exertional dyspnea for 1 year.
On examination corneal irregularities were demonstrated by rose bengal staining consistent with abnormal
From the Departments of Internal Medicine and Pathology,
University of Manitoba, Winnipeg, Canada.
Thomas Hunter, MB, BS: Assistant Professor, Department of
Medicine; Janet E. Arnott, MD: Assistant Professor of Pathology;
Dona1 S. McCarthy, MD: Associate Professor, Department of Medicine.
Addres reprint requests to Dr. Thomas Hunter, University
of Manitoba Rheumatic Disease Unit, 800 Sherbrook Street, Winnipeg, Manitoba R3A IM4, Canada.
Submitted for publication July 3, 1979 accepted in revised
form November I , 1979.
Arthritis and Rheumatism, Vol. 23. No. 3 (March 1980)
tear production. Late inspiratory rales were auscultated
at both lung bases. Musculoskeletal examination
showed diffuse tenderness of the hands with soft tissue
swelling over the dorsum of both wrists and tenderness
of the MTP joints. Laboratory investigations included
hemoglobin 12.7 gm%, WBC 5,600/mm3,
platelet count
190,000/mm3, and ESR 85 mm/hour (modified Wintrobe). Results of the latex fixation test were negative.
Antithyroglobulin antibodies were not detected. The
fluorescent antinuclear antibody test (FANA) was positive with a diffuse pattern. Multiple sputum cultures for
acid fast bacilli and fungi gave negative results.
Lung function tests showed a reduction in lung
volumes. The total lung capacity was 68% of predicted
and the vital capacity was 73% of predicted. The airflow
rates were preserved with a forced expiratory volume in
one second (FEV,) 78% of predicted and an FEV, to vital capacity ratio of 0.88. The transfer factor for carbon
monoxide measured by the single breath test was 7.7
ml/mmHg with a predicted value of 12.9 ml/mmHg.
The patient was mildly hypoxic at rest (arterial oxygen
tension 65 mmHg). Lung compliance was markedly reduced to 0.07 liters/cm H,O (predicted 0.2 liters/cm
H,O), indicating increased lung elastic recoil. These values are compatible with moderately advanced pulmonary fibrosis or interstitial lung disease and did not appreciably change after corticosteroid therapy or during
8 years of observation.
Radiographs of the chest showed bilateral hilar
lymphadenopathy and multiple small reticulonodular
densities scattered throughout both lung fields. A right
open lung biopsy was performed, and both lung and hilar lymph nodes were found to be studded with small
noncaseating granulomas (Figure 1 ). Cultures and special stains were negative for acid fast baciUi and fungi.
Figure 1. Lung showing several noncaseating granulomas with slight surrounding fibrosis ( H
On the basis of the pulmonary histology, a negative tuberculin test (5 TU), and the absence of a history of exposure to beryllium, the patient was diagnosed as having pulmonary sarcoidosis. Treatment with prednisone
40 mg/day for 6 weeks showed no improvement in the
radiographic or pulmonary function abnormalities, and
the prednisone was gradually reduced to 10 mg on alternate days.
In 1977 at age 60 years she was referred to the
Rheumatology Clinic because of persistent joint pains,
at which time her medications were prednisone 10 mg
on alternate days and thyroxine 0.2 mg daily. Examination revealed tenderness and soft tissue swelling of the
wrists, first through third MCP joints, and all the PIP
joints bilaterally. The remainder of the examination
showed no changes. Investigations included hemoglobin
12.3 gm%, WBC 5,20O/mm', ESR 35. Latex fixation
was negative, LE cell preparation was positive, FANA
strongly positive, antiDNA binding 69.5% (normal
range up to 7%, modified Farr technique) (7). The third
component of complement (C3) was 88 mg% (normal
range 120-170 mg%). The direct Coombs test was positive. The SGOT was 98 IU/liter (normal < 50), alkaline
phosphatase 278 IU/liter (normal < 125). Antismooth
+ E, X 25).
muscle and antimitochondrial antibodies were not detected. The liver scan showed nonhomogeneity of uptake of isotope suggesting hepatocellular dysfunction. A
liver biopsy was normal. Urinalysis and 24-hour urinary
protein were normal. The creatinine clearance was 67
ml/min. An intravenous pyelogram gave normal results.
The patient was followed over the next 2 years
during which her polyarthritis responded to nonsteroidal antiinflammatory drugs.
Laboratory investigations at age 62 years showed
FANA 1 :5 12, LE cell preparation positive, antiDNA
binding 65.3%, C3 70 mg% (normal range 9 4 2 1 4 mg%)
C4 8 mg% (normal range 20-50 mg%). Direct Coombs
test was positive; serum cryoglobulins were present in
trace amounts. Immunoglobulin quantitation was normal. The SGOT was 58 IU/liter and alkaline phosphatase 160 IU/liter. The prothrombin time was normal
but the partial thromboplastin time was prolonged at
85.5 seconds, control 40.5 seconds. A lupus inhibitor to
coagulation was also demonstrated. Skin tests to tuberculin (5 TU), Cundidu, and trychophyton gave negative results. The creatinine clearance was 70 ml/minute
and urinalysis normal. Radiographs of the hands and
feet showed normal findings. Radiographs of the chest
were unchanged.
Discussion. In the absence of tuberculosis, fungal infection, exposure to beryllium or other drugs or
agents that might give rise to pulmonary granulomas,
the pulmonary histology seen in this patient would be in
keeping with a diagnosis of sarcoidosis. The presence of
similar granulomas in a lymph node is also fairly characteristic of sarcoidosis. The keratoconjunctivitis sicca
and chronic nondeforming polyarthritis might also be
attributed to sarcoidosis (8- 12). Antinuclear antibodies
have been demonstrated in sarcoidosis, and the incidence of this finding has varied from that seen in normal controls (13) to 32% (14). To our knowledge antibodies to double-stranded DNA have not been detected
in sarcoidosis ( 15).
The finding of hair loss, the persistent presence
of antibodies to double-stranded DNA, along with
hypocomplementemia, positive Coombs test, and a
lupus inhibitor of coagulation support the diagnosis of
systemic lupus erythematosus. Indeed, except for the hilar lymphadenopathy and widespread pulmonary granulomas, all the manifestations of her illness are in keeping with the diagnosis of systemic lupus erythematosus.
We have found only two previous reports of patients with clinical features of both systemic lupus erythematosus and sarcoidosis (16,17). This association is
of interest in that autoantibodies, altered T lymphocyte
function, and circulating immune complexes are common to the pathogenesis of both systemic lupus erythematosus and sarcoidosis (18-23). It is possible that
the primary disease, whether systemic lupus erythematosus or sarcoidosis, predisposed to the development of
the second disease by virtue of inhibition of T lymphocyte function.
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features, lupus, occurring, systemic, erythematosus, sarcoidosis, together
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