Siliconosis : a spectrum of illness

Abstract The information reported at this symposium presents initial data supporting the notion that patients with exposure to silicone gel are experiencing a new illness. Previously, this disorder was described variously as "human adjuvant disease" and chronic silicone arthropathy. 15 During the symposium, the term siliconosis and silicone implant syndrome (SIMS) was used to describe the clinical syndrome associated with patients receiving silicone gel in liquid or encapsulated form. I prefer the term siliconosis because it is inclusive of symptomatic women who were exposed to silicone gel by injection or implant placement. Siliconosis is characterized by a spectrum of illness that may affect a number of organs. In its most common form, siliconosis is a musculoskeletal pain syndrome characterized by overwhelming fatigue, arthralgias, and myalgias. This form of the illness is reminiscent of a chronic flulike state. Influenza patients have overwhelming fatigue, fever, myalgias, and arthalgias. Common laboratory tests, such as complete blood count, erythrocyte sedimentation rate, and ANA are normal in individuals with influenza. It is difficult to document the presence of the virus, although the disease is diagnosed by physicians in the absence of positive viral cultures. Release of immune mediators (cytokines) by mononuclear cells is the most likely mechanism for the presence of constitutional symptoms. A similar circumstance may occur with the release of cytokines by mononuclear cells ingesting silicone. The criteria for classification of fibromylagia include generalized pain in symmetrical areas of the body including those above and below the waist. 29 Also included is pain with palpation in 11 of 18 tender point sites. Other factors associated with fibromylagia include nonrestorative sleep, daytime fatigue, chronic headache, and irritable bowel syndrome. Criteria for chronic fatigue syndrome include persistent fatigue sufficient to reduce daily activity by 50% or more for a period over 6 months, along with chills, cervical lymphadenopathy, headaches, myalgias, sleep disturbance, and fever. 30 Patients with silicone breast implants have similar but not identical symptoms associated with these other disorders. The severity of fatigue and the presence of a limited number of tender points differentiates this illness from fibromyalgia and chronic fatigue syndrome. Patients with fibromyalgia associated with siliconosis do not have symmetrical tender points. Most tender points are located above the waist, and In addition to the musculoskeletal symptoms associated with siliconosis, a minority of patients also experience skin rash, alopecia, fever, lymphadenopathy, and arthritis. A significant number of patients develop a Sjogren's-like disorder characterized by xerophthalmia and xerostomia. However, there is an absence of autoantibodies and cellular infiltrates associated with classic Sjogren's syndrome. Systemic lupuslike syndrome may appear with arthralgias, skin rash, and fever in the absence of detectable ANA. Rheumatoid arthritis-like disease may be characterized by years of polyarthritis without significant erosions or periarticular osteopenia. Neurological symptoms in the form of shortterm memory loss, paresthesias, and neuropathy have been reported. 31 The pathogenesis of this disorder remains to be determined. The report of Kossovsky suggests that the alterations in plasma proteins facilitate the ingestion of silicone by macrophages that initiate an immunological response characterized by a chronic inflammatory state that is associated with the production of autoantibody and cytokines. The capsule surrounding implants is a potential site for production of a number of immunologically active factors that could result in patient symptoms. Inflammatory reaction, including lymphocytes, monocytes, and plasma cells, has been identified in implant patients, with or without overt leakage. 32 Increased amounts of interleukin 2 have been detected in capsules surrounding breast implants. 33 The presence of these factors may explain the reason for the presence of the syndrome in patients with intact implants. Clinical symptoms associated with this pathological immunological state may occur in genetically predisposed patients. Preliminary studies suggest that genetically predisposed patients may have the DR53 haplotype. 34 Patients whose implants were removed have reported improvement in symptoms. The improvement is not immediate in most patients. Some patients report improvement that has its onset 12 months or more after explantation. This time course of improvement does not follow a course expected with placebo effect. One would predict that improvement with explantation would be immediate with placebo effect. Explantation of the implants, along with the surrounding capsule, removes a large amount of silicone from the patient. Improvement over 12 months or longer suggests that an abnormal immune response is decreasing with removal of the silicone. The amount of silicone that remains in patients with enlarged axillary lymph nodes may not be adequate, over time, to sustain the abnormal immune response. In some patients, the residual amounts of silicone may be adequate to sustain the pathological process. This group may contain the third of patients who do not improve with explantation. Finally, the current controversy involving silicone breast implants seems reminiscent of the debate surrounding the original patients with Lyme disease. The physicians who saw these first patients preferred to diagnose juvenile rheumatoid arthritis, a disease that they knew. Superficially, the illness seemed to be juvenile rheumatoid arthritis because it occurred in children and caused arthritis. However, closer scrutiny revealed that the illness occurred in clusters and affected adults as well as children. The illness was not juvenile rheumatoid arthritis, but a new illness caused by Borrelia burgdorferi and transmitted by a tick ( Ixodes dammini ). Our current understanding of silicone breast implants is that silicone is not inert and has the capability of causing systemic tissue damage. The mechanism by which this agent results in a clinical disease that differs from other rheumatic disorders remains to be determined. The participants of this symposium hope that the data presented will heighten interest in this condition and result in additional studies to further define the disease and improve therapies for women who are symptomatic from silicone breast implants.