Pyloric stenosis in a patient with progressive systemic sclerosis

Summary A 64-year-old Japanese woman with progressive systemic sclerosis (PSS) who developed severe pyloric stenosis is described. The conservative treatments brought only the temporary symptomatic relief, and pyloroplasty became necessary. No ulcerative lesions or tumors were found in the resected stomach or duodenum specimens implicated for stenosis. The histological examinations revealed edema and replacement fibrosis in the pyloric ring. The possible mechanisms of pyloric stenosis are discussed.     

Bioavailability of D-penicillamine in a patient with gastrointestinal progressive systemic sclerosis

D-penicillamine pharmacokinetics were studied in a patient with gastrointestinal progressive systemic sclerosis possibly complicated by malabsorption. D-penicillamine bioavailability was examined after oral, duodenal, intravenous and rectal administration. No D-penicillamine was detectable in plasma after administration to the gastrointestinal tract. The pharmacokinetics after intravenous administration agreed closely with the corresponding situation in healthy volunteers.     

Possible anti-metastatic effect of Iloprost in a patient with systemic sclerosis with lung cancer: a case study

Systemic Sclerosis (SSc) is a chronic disease of unknown etiology which affects the vascular system and connective tissue. A wide series of studies showed an increased prevalence of cancer in patients with SSc than the normal population. Prostacyclin (PGI2) is an endogenously produced element that is basically synthesized by arachiodonic acid through prostacyclin synthesis in vascular system endothelial cells. Iloprost is a stable analogue of PGI2 which is used in the treatment of pulmonary arterial hypertension (PAH). In a limited number of animal models, the anti-metastatic activity of PGI2 is observed. Herein, we report iloprost treatment of a 60-year-old-woman with SSc, who lately developed PAH as a complication of her disease and lung adenocarcinoma as a co-incidence simultaneously. These two mortal complications were both treated successfully with inhaled iloprost until her death due to gastrointestinal complications of SSc.     

Excessive fibrosis of supraclavicular lymph-node granulomas in a patient with progressive systemic sclerosis

Summary The case of a 29-year-old white female with a 7-year history of typical scleroderma is presented who developed excessive fibrosis of the supraclavicular lymph nodes. After 3 years of disease, firm right supraclavicular lymphadenopathy appeared, accompanied by a high fever. Biopsy revealed non-caseating granulomas and short-term antituberculous therapy was ineffective. The symptoms finally responded to steroids, but adenopathy persisted. A second biopsy, 40 days after the first, disclosed a similar picture with some degree of fibrosis of the granulomas. Four years later, with stony hard right supraclavicular adenopathy persisting, a third biopsy showed excessive fibrosis of the granulomas within the node and destruction of its architecture. It is postulated that the primary disease of this patient might be responsible for this clinical picture. The present seems to be the first report of such a case in the literature.     

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.     

Microscopic polyangiitis in a patient with systemic sclerosis

The coexistence of systemic sclerosis (SSc) and vasculitis has been infrequently reported. We present a 65-year-old man who, 6 years previously, had been diagnosed with limited SSc, and who developed a focal segmental necrotizing crescent glomerulonephritis associated with perinuclear antineutrophil cytoplasmic antibodies with antimyeloperoxidase specificity in the absence of Dpenicillamine exposure.     

Hemiarthroplasty in a patient with severe systemic sclerosis

Systemic sclerosis (scleroderma) is a connective tissue disorder of unknown etiology. This rare, chronic, multisystem disorder encompasses a number of variations leading to focal and systemic classifications, which includes features such as skin thickening. The changes within the skin microstructure have led to numerous surgical complications. In a review of the orthopedic literature, we found no reports describing surgical outcomes or the success of skin closures in procedures related to the hip for patients with scleroderma. We present a case of a 74-year-old man with a history of scleroderma who underwent a bipolar hemiarthroplasty to repair a right transcervical femoral neck fracture. The risk factors associated with wound healing and effects of skin contractures in systemic sclerosis patients are reviewed.     

Spontaneous pneumothorax in a patient with systemic sclerosis

Summary A 28-year-old woman developed spontaneously a right- sided pneumothorax, the leading clinical symptom of an as yet undiagnosed systemic sclerosis. The diagnosis was confirmed by Raynaud's phenomenon, microstomia, arthralgia, distal oesophageal dysfunction and antinuclear antibodies. Initial treatment with pleural suction was followed by thoracoscopy and segmental pulmonary resection. Spontaneous pneumothorax is a rare complication in patients with systemic sclerosis, most likely caused by the rupture of subpleural cysts.     

Rapidly progressive fatal interstitial lung disease in a patient with an overlap syndrome of systemic lupus erythematosus and systemic sclerosis

A 31-year-old woman with a prior history of an overlap syndrome of systemic lupus erythematosus (SLE) and systemic sclerosis (SSc) developed fever, pericarditis with pericardial effusion and a rapidly progressive fatal interstitial lung disease. Diagnostic test and procedures, differential diagnosis and therapeutic approach are discussed.     

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.     

Gastrointestinal function in patients with progressive systemic sclerosis

Summary In 24 patients with progressive systemic sclerosis (PSS) the pentagastrin-stimulated gastric acid secretion was determined to investigate if acid hypersecretion is associated with reflux-oesophagitis — the most common complication to oesophageal involvement in PSS. Gastro-oesophageal reflux was observed in 12, reflux-oesophagitis in 9 and oesophageal mycosis in 8 patients. Gastric acid secretion was increased in 13 (54%) patients and tended to be higher in patients with oesophagitis. Patients with reflux and increased acid secretion seemed to be free from oesophageal mycosis. Bacterial overgrowth and malabsorption are known complications to intestinal scleroderma and these items were investigated using non-invasive methods. Four patients had increased bile acid deconjugation, 3 had increased (¹⁴C)xylose degradation indicating bacterial overgrowth and 7 patients had decreased fat absorption in the triolein breath test. Nutritional status with respect to selenium, folate, cobalamine and fat-soluble vitamins was essentially normal.     

Disseminated intravascular coagulation in a patient with progressive systemic sclerosis associated with necrotizing angiitis and generalized lymphadenopathy.

A 47-year-old woman with progressive systemic sclerosis developed disseminated intravascular coagulation in the course of her terminal illness. She also had complicating necrotizing angiitis and generalized lymphadenopathy. The likely relationship between disseminated intravascular coagulation and vasculitis is discussed.