A 20 year old man, B.M., developed a severe inflammation of his right knee, necessitating surgery. No infection was present. Subsequent medical evaluations determined that a prior Chlamydia infection had provoked an autoimmune response, and over the next 20 years he lost mobility because of ongoing damage to his knees, ankles, toes, spine, neck and shoulders. At this point the damage wreaked by his disease began to take its toll on his internal organs. His kidneys ceased to function, being physically clogged by precipitated amyloid protein, and his spleen, four times normal size, was surgically removed. A kidney transplant ultimately failed, because of the presence of cytomegalovirus in the transplanted kidney, which the antirejection drugs allowed to proliferate and destroy its function. The individual spent his last 8 years on peritoneal dialysis, and even superficial wounds were extremely slow to heal. He died at age 50 from sepsis from the open wounds on both feet through which his heel bones protruded. He weighed 102 pounds. His initial diagnosis was Reiter's Syndrome (a condition where autoimmunity develops in response to infection in another part of the body), but the symptoms presented much later in his disease were not entirely consitent with the earlier diagnosis. Doctors began to think his condition was more consistent with symptoms of Systemic Lupus Erythematosus, however, his extreme and eventually lethal condition may not have fit any of the recognized categories of an autoimmune disorder.
This was a real individual: he had a degree in electrical engineering, and was employed by General Electric, writing computer code for safety systems for freight rail systems.
This is a touching story. I think that Chlamydia combined with some genetic predisposition and immune dysfunction might have caused the autoimmune disease. If only Chlamydia infection provoked this autoimmune disease then we are in trouble. Chlamydia infection is the most common sexually transmitted infection (STI) in U.S. The most formidable part is that this Chlamydia is asymptomatic for years and sometimes it is called “silent infection”. Not only Chlamydia causes inflammation in joints, pelvic, eye, but it also damages and kills nerve cells via apoptosis and results in degeneration of nervous system. Some autoimmune diseases can be treated with antibiotics that suppress chronic bacterial infections. Since Chlamydia is a bacterial infection, I was wondering if he was treated or could have been treated with antibiotics at initial stage of diagnosis.
ReplyDeleteI'm not totally clear on what the mechanism would have been behind his autoimmune disease. Was this a case of RA? And was he treated for the earlier Chlamydia infection? The amyloidosis suggests that the light chains of the plasma cell antibodies were aggregating in his kidneys and spleen since AL (amyloid light chain) is one of the more common mechanisms behind the buildup. As far as the dialysis goes, why did they go for the peritoneal approach? Unless he didn't have access to a more comprehensive facility, hemodialysis would have been a much more efficient way to go. This is an interesting case, I'm just becoming more curious about everything that went on here. Thanks for any insight!
ReplyDeleteB.M. was initially treated for Chlamydia. His condition first presented itself as a rheumatoid arthritic condition combined with urethritis, but over years of flare ups, increasing loss of mobility, and generally worsening health, his immune system began to attack his internal organs. Daily peritoneal dialysis was chosen as a treatment because he was so physically fragile, and the occasional hemodialysis was extremely depleting. His myriad specialists over the years did try different approaches of dialysis and medications, including Enbrel, however, ultimately, none proved effective.
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