Journal List > J Korean Rheum Assoc > v.17(4) > 1003760

Kim, Rim, Kim, Ko, Park, Park, and Jun: Systemic Sclerosis Coincidence with Sarcoidosis: A Case Report and Review of the Literature

Abstract

A 62-year-old Korean woman was admitted to our department to evaluate a chronic cough and sputum, which had begun several weeks ago. The patient had been diagnosed with systemic sclerosis in 2004. Autoantibody screening tests were negative for the anticentromere and antitopoisomerase antibodies. She received therapy with combined cyclophosphamide, a calcium channel blocker, D-penicillamine, and low dose steroid. In 2006, a pulmonary function test (PFT) showed a restrictive pattern, and a computed tomography (CT) scan of the lungs revealed interstitial lung disease, but no symptoms were present, so we maintained her on the medication. In October 2008, a chest x-ray and CT scan of the lungs demonstrated aggravation with bilateral basal interstitial infiltrates and hilar lymphadenopathy. Cyclophosphamide pulse therapy was conducted six times during 6 months, but there was no change on her chest CT and PFT, and she had no symptoms, so we decided to follow up. On admission, no significant interval change in the reticular opacity of both lower lungs was observed, but several lymph nodes were enlarged on a chest and neck CT. The skin showed multiple large polygonal-shaped scaled lesions on her upper and lower extremities. Biopsies were taken from the skin of the lower extremities and the left cervical lymph node. Typical noncaseating granulomas corresponding to sarcoidosis were found along with systemic sclerosis findings.

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Fig. 1.
Large polygonal shaped scaled lesion (white arrows) of lower extremities.
jkra-17-400f1.tif
Fig. 2.
Neck CT shows multiple lymph node enlargement (white arrows).
jkra-17-400f2.tif
Fig. 3.
Skin biopsy, showing dermal fibrosis and several noncaseating granulomas (black arrows). Granuloma consists of epitheloid cells and langerhans giant cells (Hematoxylin & Eosin stain, ×100).
jkra-17-400f3.tif
Fig. 4.
Lymph node biopsy, showing several noncaseating granulomas (black arrows). Granuloma consists of a localized collection of epithelioid histiocytes surrounded by a rim of variable numbers of lymphocytes (Hematoxylin & Eosin stain, ×100).
jkra-17-400f4.tif
Table 1.
Characteristics, radiological features, and laboratory findings of 25 patients with coexistent systemic sclerosis and sarcoidosis
References Sex subset SSc-onset Raynaud Antibody SA onset SA site Chest X-ray CT Angiotensin-converting enzyme BAL DLCO
Wiesenhutter GW3) M L ND None ILD ND
F L ND BHL ILD ND
F L ND BHL ND
Sharma OP4) F L ND None ILD ND
Enzenauer RJ5) F D T None ILD ND
Groen H1) M D 44 + T 37 BHL 91 IU/L SA→ Dec
SSc
Maekawa Y2) F L 20 C 50 Skin (mandible), LN BHL ILD 33.7 IU/L
Cox D6) F D 43 + N 44 LN, Lung BHL ILD 150 ng/mL/h Dec
M D 47 + C 59 LN, Lung BHL, Med ILD 111 ng/mL/h Dec
M L 67 + T 67 LN, Lung Rt. Hilar, Med ILD 117 ng/mL/h Dec
F L 47 + C 63 LN, Lung Pretracheal ILD 176 ng/mL/h Dec
M D 31 + T 31 LN, Lung Aortopulmonary ILD Elevated Normal
F L 43 + C 49 Lung None ILD 64 (10<n<50) Normal
F L 60 + N 60 LN, Lung Pretracheal ILD 123 ng/mL/h Dec
Arapis J7) F L 52 + T 59 LN, Lung BHL ILD 20 IU/L N Normal
De Bandt M8) F D 74 + N 76 LN, Lung
F D 29 + N 23 Lung
F D 55 + N 60 LN, Lung
M D 30 + T 32 Lung, liver, muscle, synovium
M D 52 + T 42 LN, Lung
Takahashi T9) F L 46 + ND 49 Lung BHL ILD 14.6 mg/dL (N) SA Dec
Biasi D10) F L 43 56 Lung BHL 340 nm (elevated) SA
Lis-Swiety A11) L 17 + N 20 LN, Lung BHL ND ND Normal
Tillon J12) F L 61 + C 64 Lung BHL ILD ND SA Dec
Borges da Costa J13) D 47 + T 45 LN, Lung Lung ND ND Dec

ACE: angiotensin converting enzyme, BAL: bronchoalveolar lavage, BHL: bilateral hilar lymphadenopathy, C: anticentromere antibody, CT: chest CT, D: diffuse, Dec: decreased, F: female, ILD: interstitial lung disease, L: limited, LN: lymph node, M: male, Med: mediastinal, N: negative, ND: not done, SA: sarcoidosis, SSc: systemic sclerosis, T: antitopoisomerase antibody, Blank: unknown, +: positive

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