Systemic lupus erythematosus with tibial arteritis and foot gangrene.
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Arteritis↗
Biomedical subjects
J E Ferreiro
Publications and source records attributed to J E Ferreiro.
Primary Sjögren's syndrome with diffuse cerebral vasculitis and lymphocytic interstitial pneumonitis.
A 30-year-old black Haitian man with primary Sjögren's syndrome presented with minimal sicca symptoms, negative results of serologic testing, and predominant systemic involvement, including chronic bilateral lymphoplasmocytic pulmonary infiltrates and diffuse central nervous system vasculitis. This case illustrates the recognized fact that a paucity of sicca symptoms can be overshadowed by the predominance of other systemic manifestations, delaying the diagnosis of this disease. The angiographic results in this patient have not been reported before and support the role of vasculitis in the pathogenesis of the central nervous system manifestations in primary Sjögren's syndrome.
Adult↗
Megacolon in a collagen vascular overlap syndrome.
Mixed connective tissue disease is a syndrome having clinical features of scleroderma, systemic lupus erythematosus, and polymyositis with a unique combination of clinical and laboratory findings. Not all patients with clinical features of more than one connective tissue disease fit the mixed connective disorders category; therefore, the term "overlap syndrome" is applied to patients with features of two or more connective tissue diseases. Gastrointestinal involvement in progressive systemic sclerosis is common, with the esophagus being the usual site, followed by the small bowel, colon, and stomach. Colonic involvement is reported to occur in 10 to 50 percent of cases and is typically manifested radiographically as wide-mouthed diverticular saculations. Symptoms secondary to colonic involvement are rare but occasionally serious, such as impaction of barium or feces. A case is described in which systemic lupus erythematosus coexisted with previously undiagnosed scleroderma involving predominantly the gastrointestinal tract and resulting in megacolon.
Esophagus↗
Polyarteritis manifesting as calf myositis and fever.
A patient presented with fever of undetermined cause for two months. On physical examination, bilateral calf tenderness was elicited, prompting a diagnostic muscle biopsy for polyarteritis. This limited form of polyarteritis is reviewed in the context of this case and previously reported cases. Polyarteritis limited to muscle seems to have a good prognosis and responds readily to steroids alone.
Fever of Unknown Origin↗
Thyrotoxic periodic paralysis.
A case of thyrotoxic periodic paralysis is reported in a Hispanic man with an unusual recurrence six weeks after radioactive iodine treatment. Thyrotoxic periodic paralysis has now been well characterized in the literature: it occurs primarily in Orientals with an overwhelming male preponderance and a higher association of specific HLA antigens. Clinical manifestations include onset after high carbohydrate ingestion or heavy exertion, with progressive symmetric weakness leading to flaccid paralysis of the extremities and other muscle groups, lasting several hours. If hypokalemia is present, potassium administration may help abort the attack. Although propranolol can be efficacious in preventing further episodes, the only definitive treatment is establishing a euthyroid state. The pathophysiology is still controversial, but reflects altered potassium and calcium dynamics as well as certain morphologic characteristics within the muscle unit itself.
Adult↗
Benign hypergammaglobulinemic purpura of Waldenström associated with Sjögren's syndrome. Case report and review of immunologic aspects.
Since its first description, fewer than 150 cases of benign hypergammaglobulinemic purpura of Waldeström have been reported. There is a preponderance of females with this disorder, which is characterized by long-standing purpuric vasculitic lesions usually in the lower extremities, increased sedimentation rate, anemia, leukopenia, and hyperglobulinemia with a normal clotting process. Numerous associations have been shown between this and other systemic disorders such as Sjögren's syndrome, systemic lupus erythematosus, a rheumatoid arthritis. A 40-year-old woman is described who had an 18-year history of recurrent purpura and increased IgG levels. Skin biopsy showed acute vasculitis, and immunofluorescent study revealed fibrinogen precipitation and C3 deposition. Serologic evaluation demonstrated the presence of rheumatoid factor and antinuclear antibodies (1:80). Raji assay showed increased circulating immune complexes, and the T cell subsets were normal. The purpura was associated with symptoms and physical findings of Sjögren's syndrome. On the basis of serologic and skin biopsy findings, an autoimmune origin of the disease is postulated.
Adult↗
Systemic lupus erythematosus presenting as chronic serositis with no demonstrable antinuclear antibodies.
Approximately 5 percent of patients with systemic lupus erythematosus by clinical and pathologic criteria have no demonstrable antinuclear antibodies. This figure is likely to be an underestimate, as it does not include the antinuclear antibody-negative patients with limited manifestations in whom the diagnosis of systemic lupus erythematosus is missed. A 23-year-old woman is described who had a history of perplexing bilateral pleural effusions with development of peritoneal effusion after 18 months and positive antinuclear antibody results after 22 months. Tissue pathologic features, initially interpreted as nonspecific, on review revealed striking lymphocytic periarteritis with endothelial swelling and leukocytoclastic vasculitis often seen in systemic lupus. A selective defect in the suppression of T cell effector function, such as direct cell-mediated cytotoxicity, with intact suppressor systems for B cell effector function, such as antibody production, can be postulated in this patient. This would explain the active cellular tissue pathology with the lack of prominent antinuclear antibody production.
Adult↗