Arthritis mutilans associated with cutaneous T cell lymphoma.
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Biomedical subjects
Publications and source records attributed to N Parhami.
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RHP is a recently described serum protein which inhibits a number of physiologic functions of C1q unbound to C1r2 x C1s2. In this report we show that sera from patients with rheumatoid arthritis contained elevated levels of RHP and of unbound C1q. Sera from patients with systemic lupus erythematosus contained normal levels of RHP and were characterized by deficits of C1q required to form C1 from existing levels of C1r and C1s.
We have previously shown that serum levels of C1q, unbound to C1r X C1s, are elevated in rheumatoid arthritis. We have also shown that RHP, a newly described serum protein which affects the C1q-anti C1q precipitin reaction, is also present at elevated levels in rheumatoid arthritis. We now show that RHP inhibits the hemolytic activity of C1q, disaggregates C1, and inhibits the ability of C1q bound to latex beads or to aggregated IgG to enhance the oxidative metabolism of neutrophils.
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Alveolar macrophages obtained by bronchoalveolar lavage (BAL) were evaluated by electron dispersive microanalysis (EDX) for the presence of elemental gold. EDX revealed gold in 90% (9/10) of patients with RA who were currently receiving chrysotherapy or who had discontinued chrysotherapy less than 24 months before BAL. All patients who had discontinued chrysotherapy more than 24 months before BAL (range: 3-14 years) were EDX negative (4/4), as were patients with RA who had never received gold therapy (5/5). Seven patients with RA (7/19) had clinical evidence of interstitial lung disease and 12 patients (12/19) had no interstitial lung disease. There was no correlation between chrysotherapy and the development of interstitial lung disease. These results demonstrate that gold is retained for prolonged periods in pulmonary tissue macrophages but do not identify any relationship between gold and chronic rheumatoid lung disease.
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Rheumatoid arthritis (RA), a systemic disorder of unknown cause, is associated with a variety of well-recognized pulmonary abnormalities including interstitial lung disease. To investigate possible pathogenic events in this disorder, we performed bronchoalveolar lavage (BAL) in 24 patients with classic or definite RA. Using radiographic and physiologic parameters as well as BAL cell differentials, 3 distinct groups emerged. Group I consisted of 9 patients with evidence of clinical interstitial lung involvement. Group II consisted of 5 patients without evidence of clinical interstitial lung disease (normal chest roentgenogram and functional testing) but who had abnormal BAL cellular differentials. The 10 remaining patients (Group III) had no evidence of clinical interstitial lung disease and had normal BAL cell differentials. Bronchoalveolar lavage in Group I had a significantly increased percentage of neutrophils (12.4 +/- 4.2; p less than 0.05) compared with Group II (2.4 +/- 0.8) and Group III (2.9 +/- 0.6). All patients in Group II had elevated BAL lymphocytes (24.4 +/- 6.4) compared with Group I (10.1 +/- 3.8; p greater than 0.05) and Group III (5.4 +/- 0.7; p less than 0.01). Both Groups I and II had detectable IgM in BAL (0.02 +/- 0.01 and 0.04 +/- 0.02 mg/mg of albumin, respectively), whereas Group III patients and normal control subjects did not (p less than 0.01). There was a marked reduction in BAL T-lymphocyte Leu 3/Leu 2A (helper/suppressor) cell ratios in Group I (0.92 +/- 0.02; p less than 0.05) compared with Group II (2.6 +/- 0.6) and Group III (1.6 +/- 0.1).(ABSTRACT TRUNCATED AT 250 WORDS)
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We have isolated and purified to apparent homogeneity a serum protein which appears to be a biological marker for active rheumatoid arthritis. The protein has been found in the sera in all 44 active rheumatoid arthritis patients thus far studied and is absent from, or present in undetected amounts, in sera from normal subjects or from patients with other arthritides. The protein has a molecular weight of 135,000 daltons, an isoelectric pH of 5.1-5.3, and it enhances the size of the C1q-anti C1q ring.
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All detectable C1q in serum is precipitated with the euglobulin fraction, and all other detectable hydroxyproline-containing protein in serum is excluded from this fraction. Since C1q contains 4.3% hydroxyproline, an estimate of its content in serum can be calculated by multiplying the hydroxyproline content of the euglobulin fraction by 23.3. The mean value of C1q in five normal male subjects determined by this method was found to be 63 microgram/ml as compared with 144 microgram/ml when determined by RID, indicating that the latter procedure seriously overestimates C1q in serum. C1q content of serum from patients with RA was found to be elevated by both procedures.
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We report a case of unilateral frostbite arthritis in a 58-year-old male. Clinical and radiological changes mimicked those of erosive osteoarthritis. Patients with this presentation should be carefully questioned for a history of exposure to cold, and frostbite arthritis should be considered in the differential diagnosis.
A 28-year-old man developed gangrene of a foot leading to a below-the-knee amputation. Although initially diagnosed as atherosclerotic vascular disease, clinical and laboratory findings of systemic lupus erythematosus (SLE) developed over the next 18 months. Histologic review of popliteal and femoral artery specimens showed acute and chronic changes consistent with the vasculitis of SLE. Subsequent treatment with prednisone controlled the vasculitis as well as the other clinical manifestations of SLE. The diagnosis of SLE should be considered in patients with large vessel peripheral vascular disease who lack the typical findings of the more common predisposing causes.
Clq levels in sera of adult patients with rheumatoid arthritis (RA) were found to be significantly higher than corresponding levels of normal subjects (p less than 10(-3)) The increase in Clq observed in RA was not seen in systemic lupus erythematosus, chronic polyarticular gouty arthritis, ankylosing spondylitis, Paget's disease of bone, or Hodgkin's disease. Clq levels were determined both chemically (calculated) from the protein-bound hydroxyproline content of the euglobulin fraction) and by radial immunodiffusion. Although the estimates of Clq by these 2 methods did not agree, the increase of Clq in RA was found by the use of either method.