A 44-Year-Old Woman With Polyarthritis, Fever, and Hilar Adenopathy: Conclusions
Some but not all lymphoid cells have Epstein-Barr virus-encoded RNA. Adult-onset Still disease affects women and men in the age range of 30 to 50 years. Fever can be present before the arthritis. The arthritis is often polyarticular and involves the knees, wrists, and ankles. Salmon-colored or pink macules appear on the trunk and extremities and become more prominent when patients are febrile. Patients have normal or high serum complement levels, and serum ferritin levels can be extremely high. Mediastinal adenopathy has been reported in association with Still disease. The lymph nodes in patients with Still disease have four distinct histologic patterns. These include paracortical hyperplasia with vascular proliferation and reactive lymphocytes, paracortical hyperplasia with massive sinus histiocytosis, patchy infiltration of large T-immunoblasts with high mitotic activity, and distinct follicular hyperplasia. comments
The histology can change during the course of disease and presumably reflects an abnormal immune response. The diagnosis of adult-onset Still disease is one of exclusion. The hereditary periodic fever syndromes can cause repeated attacks of fever associated with arthritis. The episodes last from 1 to 7 days. The joint involvement ranges from a monoarthritis to a pauciarticular arthritis to a symmetrical polyarthritis. These attacks are typically associated with abdominal pain and cutaneous reactions. Patients with familial Mediterranean fever and tumor necrosis factor receptor-associated periodic syndrome often have pleurisy. Patients with hyper-immunoglobulinemia D syndrome may have prominent lymphadenopathy during the attack. The high IgD syndrome is recessively inherited and is defined by a persistent polyclonal elevation of IgD. It predominantly affects northern Europeans. Arthritis in this syndrome tends to involve large joints (knees and ankles), and the synovial fluid usually has a predominance of neutrophils. The lymphadenopathy in these patients reflects an acute-phase benign hyperplastic process; splenomegaly frequently occurs in children. Finally, significant hilar and mediastinal lymphadenopathy occasionally occurs in patients with Wegener granulomatosus.
Category: Respiratory Symptoms
Tags: erythema nodosum, granuloma, lofgren syndrome, sarcoid arthritis