Frank R. Schmid

A hypocomplementemic vasculitic urticarial syndrome: Report of four new cases and definition of the disease

We describe four new patients with a unique syndrome of persistent urticaria, with leukoclastic angiitis, severe angioedema, occasional life-threatening laryngeal edema, arthritis, arthralgia, neurologic abnormalities and pronounced persistent hypocomplementemia. The complement abnormalities involved markedly reduced levels of the Clq subunit of the first component of complement (Cl) in the presence of near normal levels of Clr and Cls subunits of Cl; modest to marked depletion of the fourth component of complement (C4), the second component of complement (C2) and the third component of complement (C3); and normal levels of the fifth through ninth components of complement (C5 through C9) and properdin factors B and D. A striking serologic abnormality found in all patients was the presence of low molecular weight (7S) proteins which precipitated with Clq in agarose gels; these previously were shown to be comprised at least in part of immunoglobulin G. The present experience is offered to help to define the clinical, histopathologic and serologic characteristics of this entity, designated hypocomplementemic vasculitic urticarial syndrome, and to emphasize its distinctiveness and prevalence.

Alpha1-Antitrypsin Deficiency with Severe Panniculitis: Report of Two Cases

Two patients with profound decrease of alpha1-antitrypsin (PiZZ) presented with severe pannicultis (Weber-Christian disease); one had systemic panniculitis including pancreatitis. Another possible case is quoted from the literature. Although milder forms of panniculitis can have normal Pi phenotypes and alpha1-antitrypsin levels, the marked reduction of antiproteolytic activity found in PiZZ homozygotes may predispose to or aggravate the lesions of Weber-Christian disease.

Remission of Procainamide-Induced Lupus Erythematosus with N-Acetylprocainamide Therapy

Excerpt We describe here the case of a patient with a procainamide-induced systemic lupus erythematosus-like reaction whose arthralgias improved during antiarrhythmic therapy with the major metabol...

Arterial thrombosis in scleroderma

Clinical and pathological observations are presented on five patients with scleroderma who developed Thrombosis of a major vessel. Three died following intestinal infarction or limb gangrene and two had digit or extremity amputations. Severe intimal thickening of major arteries with thrombus formation was noted but no significant abnormality was found in the clotting activity of the blood. Histochemical studies in three cases suggested a reduction of fibrinolytic activity at the site of thrombosis in the diseased vessels. These studies support the view that the vascular system is profoundly altered in scleroderma and may play an important role in some manifestations of the disease.

Long-term antiarrhythmic therapy with N-acetylprocainamide.

The effects of long‐term NAPA therapy were evaluated in 6 patients with chronic PVCs known to respond to this drug during a previous placebo‐controlled, dose‐ranging trial. Underlying cardiac status was evaluated every six months by switching each patient from NAPA to placebo. Placebo period PVC frequency after one year of NAPA therapy was reduced, compared to baseline placebo values. Mean PEP/LVET, measured while the patients received placebo, was elevated at the beginning of the study but was normal after one year of NAPA therapy. Comparison of NAPA and placebo period observations indicated a reduction in PEP/LVET when NAPA therapy was begun. This effect, however, could not be demonstrated one year later when mean placebo period PEP/LVET was normal. The apparent dependence of this effect on underlying status of left ventricular function suggests that the initial reduction in PEP/LVET represents an indirect effect of NAPA rather than a direct inotropic action. NAPA therapy was well tolerated by the 6 patients and ANA titers became abnormal in only one, in marked contrast to reported experience with procainamide.

Effects of diflunisal on platelet function and fecal blood loss

The effects of diflunisal, a nonacetylated difluorinated salicylate, on platelet function were compared with those of aspirin and placebo. In a randomized, double‐blind trial, normal subjects were given diflunisal, 250, 500, or 1,000 mg twice daily; aspirin, 650 or 1,300 mg twice daily; or placebo for 8‐day periods. Diflunisal, 250 mg, had no effect on platelet function, whereas 500 mg induced minimal inhibition of collagen‐induced release of platelet serotonin, and 1,000 mg inhibited platelet malondialdehyde production, moderately prolonged template bleeding times (P = NS), and increased fecal blood loss (P < 0.05). In contrast, aspirin, 650 mg, markedly inhibited collagen‐induced platelet aggregation and serotonin release, and 1,300 mg prolonged bleeding time (P < 0.01) and increased fecal blood loss (P < 0.01). The effects of aspirin lasted for up to 5 days, whereas changes induced by diflunisal had returned to baseline 24 hr after the drug was discontinued. We conclude that in doses in the same range as those of aspirin diflunisal inhibits platelet function less.

Antiinflammatory Drugs and Gastrointestinal Bleeding: A Comparison of Aspirin and Ibuprofen

Gastrointestinal blood loss provoked by short-term and long-term therapy with aspirin and ibuprofen was compared in patients with rheumatoid arthritis. Blood loss was assessed by isotope counting of four-day stool collections after infusion of 51Cr-labeled autologous erythrocytes. After two weeks on drug or after one year on drug, aspirin consistently caused more bleeding than ibuprofen.

The composition and examination of synovial fluid

S ynovial fluid mirrors the pathology of joint diseases in much the same manner as the urine reflects processes taking place in the kidney. Its careful examination adds much to the accuracy of diagnosis and, at times, affords the only means of establishing it correctly. Much information can be obtained from volumes of fluid as little as 0.1 to 0.2 ml.; amounts that are obtainable from the temporomandibular joint.

Binding of Fibronectin to Clq; Inhibition of Binding by Aggregated IgG

Fibronectin was shown to bind to C1q using alkaline phosphatase conjugated fibronectin and C1q coated polystyrene tubes. The binding of the alkaline phosphatase conjugated fibronectin to C1q was dose dependent and inhibited by fibronectin and by the sulfated polymers heparin and chondroitin sulfate. The fibronectin interaction was inhibited only slightly by gelatin indicating that the fibronectin-gelatin interaction was different from that with C1q. Heat aggregated IgG blocked the binding of fibronectin to C1q and fibronectin inhibited the binding of aggregated IgG to C1q. These results suggest that fibronectin may be a factor affecting the determination of immune complexes in serum specimens by C1q binding assays.

Fibronectin associated with Clq in a Clq isolation procedure.

The complement component Clq, prepared by euglobulin precipitation of serum to which EDTA or EGTA had been added, contained fibronectin (FN) as detected by radioimmunoassay and immunodiffusion methods. The FN contents of the Clq preparations varied between 3 and 29% by weight of the Clq contents. Adsorptions of sera with polymerized IgG (an absorbent for Clq) in the presence or absence of EDTA removed all detectable Clq and between 12 and 95% of the FN. In a similar manner, adsorptions of sera and Clq preparations with insolubilized gelatin (to which FN will bind) reduced greatly or removed completely the FN component but also strikingly reduced the Clq contents. High salt concentration or the addition of EDTA did not alter the gelatin absorption results indicating that the association was not sensitive to high ionic condition and that Clq was equally bound as Clq or as the Cl complex. The results suggest that FN and Clq bind individually to both gelatin and IgG or that FN and Clq co-associate, accounting for removal of one component when the other is bound to its expected adsorbent.