Malignant thymoma associated with progressive systemic sclerosis

A 65-year-old man manifested certain features of scleroderma several years before discovery of malignant thymoma. Following tumor resection, the signs and symptoms of scleroderma did not improve, and the patient experienced the abrupt onset of renal failure with malignant hypertension 7 months after the operation. The scleroderma renal crisis caused terminal renal failure, which was treated by chronic hemodialysis. This is the second reported case of thymoma associated with progressive systemic sclerosis (PSS). The authors suggest that thymoma and various immunologic disorders have a common etiologic factor which has not yet been found. This case emphasizes that thymectomy frequently has little effect on the course of the immunologic disease. The implications of the association of thymoma and PSS are discussed.     

67Gallium lung scans in progressive systemic sclerosis

67Gallium lung scans were performed in 19 patients with progressive systemic sclerosis (scleroderma). Results were expressed quantitatively as the 67Gallium Uptake Index. The mean total pulmonary 67Gallium Uptake Index in patients was significantly higher than that in controls (41 versus 25), and 4 patients (21%) fell outside the normal range. There were no clinical or laboratory variables that correlated with the 56gallium uptake. Increased pulmonary 67gallium uptake in scleroderma may prove useful as an index of pulmonary disease activity.     

Atypical esophageal diverticula associated with progressive systemic sclerosis.

Five cases of unusual esophageal diverticula associated with progressive systemic sclerosis (scleroderma) or collagen vascular disease are presented. These wide-mouthed saccular diverticula were infrequently seen in a group of PSS patients with the typical motility disturbance of esophageal involvement and are reminiscent of the diverticula of the colon involved by PSS.     

Corrugated mucosal pattern in the esophagus associated with progressive systemic sclerosis

Three cases of unusual corrugated mucosal pattern in the esophagus accompanying the typical motility disturbance of progressive systemic sclerosis (PSS) are reported. The changes were rarely seen in a group of patients with esophageal involvement by PSS.     

Unexplained systemic symptoms and Gallium-67--guided decisions

Over 3 months, a healthy man developed prominent systemic symptoms that defied investigation. Physical examination was noncontributory, and extensive studies revealed only a marked acute-phase response associated with increased serum IL-6 levels. A whole body Gallium-67 scan was crucial in diagnosis, directing attention to high uptake in the left paraspinal and psoas muscles. Open surgical excision biopsy was performed, guided by intraoperative use of a gamma-probe. Removed tissue was diagnosed as diffuse, large B-cell non-Hodgkin lymphoma of muscle (stage IE), a rare extranodal lymphoma. Cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) therapy was given, and the patient became asymptomatic with normal blood tests and was thought to be in remission. However, a repeat Gallium-67 scan revealed recurrent multifocal disease and salvage chemotherapy was instituted. A 47,XXY karyotype (Klinefelter syndrome) was later identified, possibly associated with the lymphoma.     

Giant cell granulamatous hypophysitis with remarkable uptake on Gallium-67 scintigraphy

We treated a man with giant cell granulomatous hypophysitis with pituitary enlargement, as seen on magnetic resonance imaging. Endocrinological examination revealed panhypopituitarism and diabetes insipidus. Microscopic examination of the specimen obtained by transsphenoidal pituitary biopsy revealed a granulomatous lesion, composed of epitheliod cells, Langhans' multinucleated giant cells, lymphocytes and other chronic inflammatory cells. On whole body gallium-67 scintigraphy, there was extensive uptake in the pituitary gland. Gallium-67 scintigraphy may greatly aid in the diagnosis of granulomatous hypophysitis.     

L-tryptophan ingestion associated with eosinophilic fasciitis but not progressive systemic sclerosis

OBJECTIVE: To assess the use of L-tryptophan by patients with eosinophilic fasciitis and compare this with its use by patients with progressive systemic sclerosis (scleroderma). DESIGN: Retrospective and prospective analysis. Six patients with eosinophilic fasciitis were identified retrospectively and two prospectively. Retrospective identification of patients was done by questioning hospital-affiliated rheumatologists and dermatologists and by searching the hospital dermatopathology database. The patients with scleroderma or morphea were prospectively identified by questioning consecutive office patients with these established diagnoses. SETTING: University of Pennsylvania rheumatology and dermatology practices. PATIENTS: Eight patients with eosinophilic fasciitis; 40 consecutive patients with scleroderma (27 with diffuse cutaneous and 13, limited cutaneous disease); 3 patients with morphea. RESULTS OF DATA ANALYSIS: All eight patients with eosinophilic fasciitis had taken L-tryptophan before the onset of their disease. All had myalgias and high peripheral eosinophil counts (most greater than 5000 cells/mm3). Only 1 of 40 patients with scleroderma (no patients with morphea) had used L-tryptophan preceding illness (P less than 0.001 compared with eosinophilic fasciitis). Six patients with eosinophilic fasciitis had taken L-tryptophan for less than 8 months. One patient had taken it for 9 years before developing skin induration. Two patients were newly identified as having hypothyroidism; two developed neuropathy; and two had severe flexion contractures (several occurring in areas without skin induration). Five patients had low-titer antinuclear antibodies, indicating a possible autoimmune process. Most patients had only a partial response to systemic corticosteroid therapy. One patient has had important disease regression in response to isotretenoin therapy that was evident even while she continued to take L-tryptophan. CONCLUSIONS: L-Tryptophan use can lead to eosinophilic fasciitis whereas it does not appear to cause classic scleroderma. The disease process does not automatically remit after discontinuation of L-tryptophan-containing products.     

Conjugal progressive systemic sclerosis

INTRODUCTION: Familial occurrence of progressive systemic sclerosis is unusual. The occurrence of conjugal scleroderma is exceptional. EXEGESIS: We report here a case of systemic sclerosis in a wife and husband who both developed the onset of illness within a 10-year period. Solvent exposure was noted. CONCLUSION: The etiology of systemic sclerosis remains unknown. Environmental factors may play role in its pathogenesis.     

Multiple sclerosis associated with systemic sclerosis

Multiple sclerosis (MS) has been increasingly reported in association with other autoimmune diseases not primary affected the nervous system. The coexistence of MS and systemic sclerosis (SSc) has been rarely described. We report here the case of a 46-year-old female patient with longstanding MS since the age of 26, who developed SSc 12 years later. Her MS was of the relapsing-remitting type, but the definite diagnosis was not made until the age of 33. MS diagnosis was based on medical history, magnetic resonance imaging (MRI) studies and positive cerebrospinal fluid analysis. One year after the diagnosis of MS, she developed Raynaud’s phenomenon, skin tightness and hypopigmented patches, suggestive of scleroderma. Further investigation with laboratory studies, including serology, hand and chest X-rays, and chest computerized tomography scan confirmed the SSc diagnosis. Our report highlights the interesting association between MS and SSc that may be due to an overlapping pathogenetic mechanism for both processes.