Optic neuropathy and central nervous system disease associated with primary Sjögren's syndrome.

Three cases of optic neuropathy associated with primary Sjögren's syndrome are reported. All three patients had clinical manifestations of primary Sjögren's syndrome, although two of the patients did not report sicca symptoms at initial examination. Two patients had focal neurologic signs in addition to optic neuropathy. The differentiation of this syndrome of optic neuropathy, focal neurologic signs, and Sjögren's syndrome from multiple sclerosis and antiphospholipid antibody syndrome is important for reasons of treatment and prognosis. This diagnostic differentiation was facilitated by positive tests for xerophthalmia and findings of positive minor salivary gland biopsy. High titers of antinuclear antibody, anti-SSA(Ro), and anti-SSB(La), and the absence of antiphospholipid antibodies provided additional help in the differential diagnosis. In 5 years of observation, none of the patients developed symptoms of multiple sclerosis or additional connective tissue disorders.

The frequency of sicca syndrome in an elderly female population.

In a population of 103 elderly Caucasian women, 39% had sicca symptoms and 24% had abnormal Schirmer's tear tests. Two (2%) satisfied criteria for Sjögren's syndrome and 12 (12%) were felt to have possible Sjögren's syndrome. Fifty percent of those with sicca were taking medications that might induce mucosal dryness. Fibrosis was noted on all 23 labial minor salivary gland biopsy specimens obtained. Mucosal drying medications and senile salivary gland atrophy seemed to contribute to the high frequency of sicca in this population with a lesser proportion of the subjects demonstrating previously undiagnosed Sjögren's and possible Sjögren's syndrome.

Pulmonary vascular response during Raynaud's phenomenon in progressive systemic sclerosis.

Progressive systemic sclerosis and Raynaud's phenomenon are associated with a distinctly vascular form of pulmonary involvement and pulmonary hypertension. To investigate a possible underlying vasospastic predisposition in these patients, the pulmonary vascular response to Raynaud's phenomenon induced by cold-water hand immersion was examined in nine patients. Four patients had pulmonary fibrosis and four patients had the CREST syndrome; no patient had pre-existing pulmonary hypertension. During Raynaud's phenomenon, there was no significant rise in mean pulmonary artery pressure (15 +/- 3 versus 15 +/- 2 mm Hg, p = NS) or pulmonary vascular resistance (112 +/- 38 versus 118 +/- 50 dynes X second X cm-5, p = NS) over baseline, despite a significant rise in mean aortic pressure (104 +/- 14 versus 92 +/- 11 mm Hg, p less than 0.01) and systemic vascular resistance (1,700 +/- 450 versus 1,500 +/- 470 dynes X second X cm-5, p less than 0.01). It is concluded that pulmonary vasospasm with transient pulmonary hypertension does not occur in patients with progressive systemic sclerosis and Raynaud's phenomenon during episodes of Raynaud's phenomenon. Abnormal pulmonary vasospasm in these patients in response to other stimuli, however, is not excluded.

Dermatomyositis and dermatitis herpetiformis.

Primary hypothyroidism, dermatomyositis, dermatitis herpetiformis (DH), and Sjögren's syndrome developed sequentially, over a 20-year period, in a 67-year-old woman. The principal manifestations of her illness were periodic and simultaneous exacerbations of dermatomyositis and DH. Her histocompatability antigens were A1,B8, a haplotype associated with autoimmune diseases. We suggest that this unusual disease complex has a genetic basis.

Hypokalemic periodic paralysis in Sjögren's syndrome.

A 30-year-old woman had scleroderma, Sjögren's syndrome, deforming polyarthritis, distal renal tubular acidosis, hypokalemic periodic paralysis, and persistent mild myopathy. During a five-year period the patient's otherwise mild course of disease was complicated by the occurrence of five episodes of severe flaccid muscle paralysis involving both proximal and distal muscle groups. Between the paralytic episodes the patient functioned well without replacement therapy, and had normal potassium levels. The sicca component was mild and went unrecognized for several years. There was no family history of muscle disease. The data presented in this report support the view that the paralytic episodes were due to hypokalemia secondary to renal tubular acidosis associated with Sjögren's syndrome. Hypokalemic periodic paralysis may occur as a rare complication of Sjögren's syndrome and renal tubular acidosis.

The role of IgM rheumatoid factor in experimental immune vasculitis.

The effect of IgM rhematoid factor (RF) on reversepassive cutaneous Arthus reaction in rats was studied. The RF was obtained from the serum cryoglobulin of a patient with symptoms of purpura, arthralgia and digital gangrene. The cryoglobulins was of IgG-IgM type and when given i.v it induced a prompt hypocomplementaemia in experimental animals. The purified RF also induced low serum complement levels when injected i.v. along with complexes of non-complement-fixing, aggregated IgG. A reverse passive Arthus reaction was induced by intradermal injection of IgG anti-bovine serum albumin (BSA), followed by an i.v. dose of antigen (Ag). The cutaneous inflammatory reaction was aggravated by simultaneous administration of IgM RF intradermally, but not by IgM without antibody (Ab) properties. Intradermal injection of low concentrations of non-complement-fixing IgG anti-BSA, along with normal human IgM, followed by i.v. injection of BSA, resulted in a complete lack of cutaneous inflammation. At higher Ab concentrations there was only a mild inflammation. However, when IgM RF was substituted for normal IgM and injected with non-complement-fixing anti-BSA, an effective reverse passive cutaneous Arthus reaction and vasculitis was induced. The inflammatory response was greatly suppressed by decomplementation of animals by cobra venom factor. This study provides evidence favouring an inflammatory, complement-dependent role for RF in vasculitis.

Hormone storage granules in the beef anterior pituitary. I. Isolation, ultrastructure, and some biochemical properties.

A method is described for isolation of relatively large quantities of large and small hormone storage granules from the beef adenohypophysis. The hormone storage granules are highly purified, as indicated by ultrastructural and biochemical criteria. The average size of large granules is 400 mmicro and of small granules is 220 mmicro. The large granules contain growth hormone and prolactin; the small granules contain high concentrations of follicle-stimulating, luteinizing, and thyroid-stimulating hormones. An alkaline protease with a pH optimum of 8.3 is associated with the small granule fraction.