Popliteal artery thrombosis in a patient with Cogan syndrome: treatment with thrombolysis and percutaneous transluminal angioplasty.

A 31-year-old woman with Cogan syndrome (a rare form of systemic vasculitis) was evaluated for a cold, painful left foot with diminished pulses. Arteriography demonstrated thrombosis of the left popliteal artery with evidence of vasculitis. Thrombolytic therapy was begun with initial success but eventual rethrombosis. After reinitiating thrombolytic therapy combined with intraarterial vasodilator therapy, successful angioplasty was performed with sustained results at 6-month follow-up.

Giant cell arteritis and polymyalgia rheumatica.

The close relationship between giant cell arteritis and polymyalgia rheumatica has not been clearly explained. These disorders affect the same patient population and often coexist in the same person. Monitoring of the erythrocyte sedimentation rate is a useful tool in both diagnosis and treatment. Management with varying doses of prednisone has proved effective in resolving symptoms.

Rethinking the therapeutic pyramid for rheumatoid arthritis. When are NSAIDs alone not enough?

A large number of agents that compare quite favorably to nonsteroidal anti-inflammatory drugs in terms of toxicity and efficacy are available to physicians for the treatment of rheumatoid arthritis. Primary care physicians need to familiarize themselves with the use of these drugs and consider prescribing them early in the course of the disease, when they may be of greatest benefit.

Anti-histone antibodies in the serum of autoimmune MRL and NZB/NZW1 F1 mice.

Anti-histone antibodies have been reported in a number of human autoimmune diseases, most notably idiopathic and drug-induced lupus erythematosus. In the current study, anti-histone antibody activity was detected using ELISA and electroblotting techniques in sera from autoimmune NZB/W, MRL-lpr, and MRL-(+)/+ mice. Anti-histone activity increased with age, maturing earlier in females, in both NZB/W and MRL-lpr mice. Testosterone treatment decreased anti-histone activity in NZB/W mice and estrogen treatment from 2 weeks of age increased anti-histone activity in MRL-lpr mice, suggesting that gonadal hormones modified the expression of autoantibodies recognizing these protein antigens. Estrogen also increased serum IgG levels in MRL-lpr mice. Sex hormones affected expression of antibodies recognizing soy milk proteins but not ovalbumin in a similar manner. Nitrocellulose Western blots of SDS gels probed with sera from both types of autoimmune mice most often demonstrated reactivity with histone1. Some mice, usually mature females, also recognized histone4, histone3, and histone2.

Prospective analysis of liver biopsies before and after methotrexate therapy in rheumatoid patients.

The significance of hepatic changes in methotrexate-treated RA patients is unclear at this time. In our group of RA patients, there was a slight increase in the incidence of triaditis and fat during methotrexate therapy. Disease duration greater than or equal to 10 years was associated with increased hepatic triaditis before treatment. Age greater than 50 years was associated with increased hepatic fat before and after treatment. It appears that patients' ages and duration of underlying RA account for some changes, independent of methotrexate therapy. Several of our patients changed from higher to lower histologic grade or had an apparent decrease in fibrosis, fat, or triaditis on the pathologists' reports and the blind readings of the repeat biopsies. This may be explained by sampling error. More importantly, some of these changes may not be of clinical significance. One report of methotrexate-induced cirrhosis in patients with psoriasis demonstrated that in all but one of 14 patients who continued receiving methotrexate the cirrhosis decrease or did not progress. This may also be true of the hepatic fibrosis seen in RA after methotrexate treatment. In this study, there did not appear to be changes seen on pretreatment liver biopsy that were predictive of subsequent fibrosis or cirrhosis. Our data indicate that pretreatment biopsy is unwarranted in a population similar to ours. However, our practice has been to try to avoid methotrexate in patients with diabetes, prior liver disease, alcoholism, or obesity because of previous reports suggesting that these patients are at increased risk for the development of cirrhosis. Only the above-mentioned patient, eventually diagnosed as having cirrhosis, might have been handled differently. Including the study, none of the approximately 700 RA patients in the literature having liver biopsies after methotrexate therapy have developed cirrhosis consequent to its use. Most of these had received a total dose of approximately 1,500 mg in small weekly doses, and alcohol was prohibited. Below this cumulative dose the risk of clinically significant liver damage in carefully selected patients is very low. In view of this experience, the recommendation that RA patients have liver biopsies after 1,500 mg of methotrexate (a holdover from the psoriasis literature) may be too conservative in low-risk RA patients, provided methotrexate is administered weekly and alcohol is prohibited. Recognizing that the absolute need for biopsy is unproven, a more realistic milestone for those choosing biopsy might be after each 2,000 to 2,500 mg.(ABSTRACT TRUNCATED AT 400 WORDS)

Inflammatory central nervous system involvement in rheumatoid arthritis.

We describe a patient with seropositive rheumatoid arthritis who developed pachymeningitis resulting in optic atrophy. Clinical, histopathologic, and radiologic findings in 18 additional cases of inflammatory CNS disease associated with rheumatoid arthritis are reviewed. The three characteristic neuropathologic findings were rheumatoid nodules, pachymeningitis or leptomeningitis, and vasculitis. In most cases, more than one of these histopathologic processes were found. The typical host was middle-aged with long-standing severe nodular disease. However, contrary to previous reports, CNS disease occurred in a significant number of patients without active synovitis and extracranial vasculitis and nodules. Although no correlation between specific neurologic symptoms and neuropathology was noted, patients with CNS nodules tended to be asymptomatic more often than patients with vasculitis or meningitis. CSF analysis and computed axial tomography were helpful diagnostic tools, but diagnosis was ultimately made only by directed biopsy or at autopsy. Treatment with surgical decompression and/or corticosteroids has proved beneficial in several cases. Inflammatory CNS involvement in rheumatoid arthritis should be considered in any patient with neurologic symptoms in whom infectious and malignant processes are ruled out. An aggressive, invasive approach for diagnostic biopsies seems warranted.

Methotrexate therapy in rheumatoid arthritis.

Rheumatoid arthritis (RA) is a common disease with a significant economic and social impact on Americans. Many patients with RA are unresponsive to or intolerant of conventional therapy or the limited therapeutic options available. For many of those patients, immunosuppressive drugs have been the mainstay of therapy. Our experience with methotrexate for these patients indicates that this drug provides symptomatic relief and improvement in objective parameters. Significant toxicity was uncommon. Methotrexate should be considered for selected patients with severe rheumatoid arthritis when conventional measures have been exhausted.

The characterization of normal and scleroderma skin fibroblasts cultured in a collagen gel matrix.

Fibroblasts from normal and progressive systemic sclerosis involved skin more closely resemble in vivo cells when cultured in a collagen gel matrix, than do fibroblasts cultured on plastic. Ultrastructural studies show that the cytoskeleton and secretory organelles from these cells are better developed in the presence of a collagen gel. Fibronectin, glycosaminoglycans and collagen fibrils are produced by the cells and deposited in the preformed matrix. Collagen synthesis is greater in the scleroderma derived fibroblasts for all culture conditions with increased deposition in the matrix. Total collagen production form cells associated with the gel is less than that from those cultured on plastic, suggesting an inhibition of collagen synthesis by the preformed collagen matrix.

Rheumatoid vasculitis with intrarenal aneurysm formation.

A 58-year-old male with an 8-year history of seropositive erosive rheumatoid arthritis (RA) presented with acute abdominal pain and syncope; a diagnosis of vasculitis was made arteriographically by finding intrarenal saccular aneurysms. Patients with severe RA and a high titer of rheumatoid factor may develop a diffuse vasculitis, but clinical renal involvement attributable to vasculitis is uncommon. Our case demonstrates the coexistence of RA and renal vasculitis, documented by autopsy findings, and for the first time by arteriographic demonstration of intrarenal aneurysm formation.

Chronic human colchicine neuropathy and myopathy.

A 43-year-old woman who indiscriminately took colchicine for five years developed a progressive multisystem syndrome that included severe axonal neuropathy and a pathologically striking myopathy. The neuropathy and myopathy were similar to experimental animal colchicine-induced neuromuscular toxicity.

Ocular chrysiasis.

Thirty-four patients who had received over 1 gram of gold compounds for rheumatoid arthritis were examined for ocular chrysiasis. Ninety-seven percent of the patients receiving continuous chrysotherapy at the time of their ocular examination exhibited corneal chrysiasis. With few exceptions, corneal gold deposits were limited to the posterior one-half of the corneal thickness. Deposits tended to concentrate inferiorly and to spare the superior and peripheral cornea. Duration of chrysotherapy correlated positively with the clinically graded density of deposits. In this series, 55% (16/29) of these patients receiving gold therapy for three years or more had lenticular chrysiasis, although this has previously been considered rare. Our data suggest that gold is deposited in the cornea and lens from the anterior chamber aqueous fluid.

Hypophosphatemia and neurological changes secondary to oral caloric intake: a variant of hyperalimentation syndrome.

Previous reports have described a syndrome of paresthesias, weakness, seizures and hypophosphatemia in patients and animals receiving intravenous hyperalimentation. In this report we describe a group of five patients who developed this syndrome while on oral caloric intake and three patients who received only modest amounts of hyperalimentation therapy. As an experimental corollary, studies were performed in starved and normal dogs with calories infused via an intragastric catheter. The serum inorganic phosphorus (Pi) fell slightly in normal animals from 4.8-2.5 mg. %. In the starved dogs with diarrhea or vomiting the Pi fell gradually from 4.8-1.6. In starved dogs without gastrointestinal symptoms the Pi fell precipitously from 3.7-1.4 mg % on the first day of infusion and remained at that level. Approximately 50% of the starved animals developed the neurological syndrome; none of the normal animals had neurological symptoms.

Transplantation in the hemolytic-uremic syndrome.

A case history of a fifty-seven-year-old white woman with hemolytic-uremic syndrome who was successfully transplanted with a cadaver homograft is reported. A review of the pertinent literature regarding the pathophysiology of the syndrome and the experience to date with transplantation is presented.