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Introduction Polyneuropathy signs (Neuropathy Impairment Score, NIS), neurophysiologic tests (m+7Ionis ), disability, and health scores were assessed in baseline evaluations of 100 patients entered into an oligonucleotide familial amyloidotic polyneuropathy (FAP) trial. Methods We assessed: (1) Proficiency of grading neurologic signs and correlation with neurophysiologic tests, and (2) clinometric performance of modified NIS+7 neurophysiologic tests (mNIS+7Ionis ) and its subscores and correlation with disability and health scores. Results The mNIS+7Ionis sensitively detected, characterized, and broadly scaled diverse polyneuropathy impairments. Polyneuropathy signs (NIS and subscores) correlated with neurophysiology tests, disability, and health scores. Smart Somatotopic Quantitative Sensation Testing of heat as pain 5 provided a needed measure of small fiber involvement not adequately assessed by other tests. Conclusions Specially trained neurologists accurately assessed neuropathy signs as compared to referenced neurophysiologic tests. The score, mNIS+7Ionis , broadly detected, characterized, and scaled polyneuropathy abnormality in FAP, which correlated with disability and health scores. Muscle Nerve 56: 901-911, 2017.

Objective To investigate whether treatment with abetimus delays renal flare in patients with lupus nephritis. Secondary objectives included evaluation of the effect of abetimus on C3 levels, anti–double-stranded DNA (anti-dsDNA) antibody levels, use of high-dose corticosteroids and/or cyclophosphamide, and major systemic lupus erythematosus (SLE) flare. Methods We conducted a randomized, placebo-controlled study of treatment with abetimus at 100 mg/week for up to 22 months in SLE patients. Three hundred seventeen patients with a history of renal flare and anti-dsDNA levels >15 IU/ml were randomized to a treatment group (158 abetimus, 159 placebo); 298 (94%) were enrolled in the intent-to-treat (ITT) population (145 abetimus, 153 placebo), based on the presence of high-affinity antibodies for the oligonucleotide epitope of abetimus at baseline screening. Results Abetimus did not significantly prolong time to renal flare, time to initiation of high-dose corticosteroid and/or cyclophosphamide treatment, or time to major SLE flare. However, there were 25% fewer renal flares in the abetimus group compared with the placebo group (17 of 145 abetimus-treated patients [12%] versus 24 of 153 placebo-treated patients [16%]). Abetimus treatment decreased anti-dsDNA antibody levels (P < 0.0001), and reductions in anti-dsDNA levels were associated with increases in C3 levels (P < 0.0001). More patients in the abetimus group experienced ≥50% reductions in proteinuria at 1 year, compared with the placebo group (nominal P = 0.047). Trends toward reduced rates of renal flare and major SLE flare were noted in patients treated with abetimus who had impaired renal function at baseline. Treatment with abetimus for up to 22 months was well tolerated. Conclusion Abetimus at 100 mg/week significantly reduced anti-dsDNA antibody levels but did not significantly prolong time to renal flare when compared with placebo. Multiple positive trends in renal end points were observed in the abetimus treatment group.

Human T cell lymphotropic virus type I (HTLV-I) gag and env protein-specific antibodies were identified in 6 of 13 patients with essential cryoglobulinemia (EC), by Western blot and radioimmunoprecipitation analysis. Supernatants of cells from 2 of the 5 EC patients tested showed reverse transcriptase activity. DNA sequences homologous to HTLV-I could not be detected by polymerase chain reaction, thus excluding the presence of prototype HTLV-I in each patient with EC. The data suggest that retroviral proteins distinct from but related to HTLV-I may be involved in the pathogenesis of EC in some patients.

Amyloid deposition in the iris is rare, scanty and associated with systemic disease. We present a case of isolated localized heavy amyloid infiltration of the iris and describe a new hillock-pattern feature visualized using scanning electron microscopy.

A prospective clinical study evaluated the effects of combined chemotherapy, steroids and plasmapheresis on 5 patients with mixed cryoglobulinemia with renal or neurologic and vasculitic manifestations. Treatment included prednisone (1mg/kg/day), chlorambucil (white blood cell count at greater than 3,000/mm3), and plasmapheresis (1 to 3 liters/week). There was healing of cutaneous ulcers (3/3), improvement in renal function (4/4), and diminution of purpura (2/2), but little improvement in peripheral neuropathy. Complications included leukopenia, perineal ulcers, and osteomyelitis.

The presence of C-reactive protein (CRP) was determined in the sera of 70 patients with systemic lupus erythematosus (SLE), 24 patients with rheumatoid arthritis (RA), and 17 patients hospitalized with acute infections. CRP (precipitin reaction > 3 mm) was present in only 2 of 50 patients with active, uncomplicated SLE (4%). However CRP was found in the sera of 11 patients with active SLE in the presence of superimposed infection. Treatment of the intercurrent infection resulted in the loss of CRP from the sera of these SLE patients. CRP was found in 85% of patients with active RA and in 100% of non-SLE infected patients. Appropriate treatment of the latter group resulted in loss of CRP from the convalescent sera. Corticosteroid therapy did not appear to influence the presence of CRP in the RA or SLE groups. Fourteen of 50 noninfected patients (28%) with active SLE were not receiving steroids at the time of CRP negativity. Appropriate experiments with mixed sera failed to demonstrate serum factors that could influence CRP precipitation in the CRP-negative SLE group. These studies suggest that patients with active SLE usually do not have CRP in their sera, and the results distinguish this group of patients from those with active RA, a disease generally marked by CRP positivity. Moreover, the finding of significant CRP precipitin in the sera of SLE patients may suggest the presence of superimposed infection.