A Case of Progressive Systemic Sclerosis Associated with Sarcoidosis and Esophageal Adenocarcinoma

Abstract

A 50-year-old Japanese female with progressive systemic sclerosis (PSS, CREST syndrome) is reported. During treatment for PSS, she was diagnosed by clinical and laboratory findings as having sarcoidosis, which was confirmed by histological examination of the skin and lymph nodes in July of 1991. She complained of back pain in August of 1991. Reflux esophagitis and Barrett's esophagus, found by endoscopy, progressed into a well-differentiated tubular adenocarcinoma. This is a very rare case of PSS associated with sarcoidosis and esophageal adenocarcinoma developing from Barrett's esophagus.

Clinical characteristics of sarcoidosis patients with systemic sclerosis‐specific autoantibody: Possible involvement of thymus and activation‐regulated chemokine and a review of the published works

“…While rare, the coexistence of sarcoidosis with SSc has been reported in the published work, and we found nine cases of cutaneous lesion of sarcoidosis described in detail (Table ). Together with our two cases (cases 1 and 3), a review of these cases revealed a predominance of females (100%), with a median age of 53 years . ACA was detected in six patients (6/11; 55%) and anti‐topoisomerase I antibody was observed in three.…”

A case of sarcoidosis developing as sarcoid myopathy concomitant with systemic sclerosis and review of the literature

“…Radiographically, 18 cases had ILD. Bilateral hilar lymphadenopathy (BHL) was found in 9 cases [2][3][4][5][6][7][8][9][10][11][12]. In our case, making the diagnosis of systemic sclerosis coincided with that of sarcoidosis, showing diffuse type of systemic sclerosis, elevation of serum ACE, and ILD without hilar adenopathy.…”

“…The association between systemic sclerosis and sarcoidosis has not been fully understood, but at least 24 cases Abbreviations are as follows: ACA anti-centromere antibody, ACE angiotensine converting enzyme, BHL bilateral hilar adenopathy, ILD interstitial lung disease suffering from both systemic sclerosis and sarcoidosis were found by review of literature [2][3][4][5][6][7][8][9][10][11][12]. The characteristics of the twenty-five cases including our case are summarized in Table 1.…”

“…In addition, some cases possessing both systemic sclerosis and sarcoidosis have been reported previously [2][3][4][5][6][7][8][9][10][11][12]. The common manifestations of sarcoidosis result from pulmonary, cutaneous, and eye involvements [13].…”

Association of Progressive Systemic Sclerosis with Pulmonary Sarcoidosis. Just a Chance Occurrence?

“…Not only progressive systemic sclerosis (PSS), but also Hashimoto's thyroiditis, idiopathic thrombocytopenia, rheumatoid arthritis , and other autoimmune disorders are reported to exist with sarcoidosis (3)(4)(5)(6)(7)(8)(9). Here, we describe a patient with interstitial pneumonia associated with PSS.…”