Systemic Sclerosis Coincidence with Sarcoidosis: A Case Report and Review of the Literature

Dong Chan Kim; Dong Hwi Rim; Young Taek Kim; Joo Yeon Ko; Chan Kum Park; Sung Soo Park; Jae-Bum Jun

doi:10.4078/jkra.2010.17.4.400

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

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

Original Article

J Korean Rheum Assoc 2010;17(4):400-405.

Published online: 23 December 2010

DOI: https://doi.org/10.4078/jkra.2010.17.4.400

Systemic Sclerosis Coincidence with Sarcoidosis: A Case Report and Review of the Literature

Dong Chan Kim¹, Dong Hwi Rim¹, Young Taek Kim¹, Joo Yeon Ko², Chan Kum Park³, Sung Soo Park¹, Jae-Bum Jun⁴

¹Departments of Internal Medicine, Seoul, Korea

²Departments of Internal Dermatology, Seoul, Korea

³Departments of Internal Dermatology and Pathology, Seoul, Korea

⁴Hanyang University College of Medicine, Department of Rheumatology, Hanyang University Hospital for Rheumatic Disease, Seoul, Korea

Received 6 April 201029 June 2010 Accepted 30 June 2010

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

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.

Keywords: Systemic sclerosis, Sarcoidosis

REFERENCES

1). Groen H., Postma DS., Kallenberg CG. Interstitial lung disease and myositis in a patient with simultaneously occurring sarcoidosis and scleroderma. Chest. 1993. 104:1298–300.

2). Maekawa Y., Nogami R. A case of progressive systemic sclerosis associated with sarcoidosis and esophageal adenocarcinoma. J Dermatol. 1993. 20:45–48.

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Fig. 1.

Large polygonal shaped scaled lesion (white arrows) of lower extremities.

Fig. 2.

Neck CT shows multiple lymph node enlargement (white arrows).

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).

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).

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	DL_CO
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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