A case report of a 33-year-old woman with progressive systemic sclerosis and polymyositis is presented. Admitted with a β-hemolyticStreptococcusinfection of the right knee joint, she had progressive systemic sclerosis with small intestinal involvement, which is rare. This led to more serious complications, malabsorption, pneumoperitoneum, and pneumatosis cystoides intestinalis, which forced a decision to treat her with home total parentral nutrition. The diagnostic as well as treatment problems encountered in this patient illustrate the importance of nursing care in the overall management of patients with this disease.