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PubMed1979

[Scleroderma with traces of disseminated lupus erythematosus associated with Hashimoto's thyroiditis and papillary carcinoma of the thyroid gland (author's transl)].

The coexistence of organ-specific and nonorgan-specific autoimmune diseases is an interesting phenomenon. A 52-year-old woman was admitted with fever, general discomfort, polyarthritis, and Raynaud's phenomenon. Physical examination revealed a goiter of stony consistency, hardening, paleness, and atrophy of the skin on the face and upper limbs, and blood hypertension (180/110 mmHg). The biological data included leukopenia, moderate anemia, and a very high sedimentation rate. The latex test was positive (+++); LE cells positive (+); hypergammaglobulinemia (3.5 g); antinuclear antibodies, 1/1280 with an immunofluorescence granular pattern; antithyroid antibodies, 1/160. There was pulmonary, renal, and gastrointestinal involvement compatible with scleroderma, which was confirmed by skin biopsy. A thyroidectomy revealed the existence of a papillary carcinoma with thyroiditis. Responde to treatment with immunosuppressive agents, hypotensive drugs, and thyroid substitution therapy was initially good. The patient was readmitted 8 months later with general discomfort and a severe hyperproteinemia (10 g/100 ml), including 65 percent gammaglobulin and requiring various sessions of plasmapheresis. The patient was discharged, but died suddenly 4 months later. The association of lupus and scleroderma in this patient is discussed and the possibility of its being a mixed connective tissue disease is discarded. The association of this condition with Hashimoto's thyroiditis, and the latter with papillary carcinoma of the thyroid are analyzed. The peculiar features of this case are pointed out. The authors postulate that the cause of the sudden death was a vascular cerebral complication induced by the extreme hyperproteinemia.

Carcinoma, Papillary↗
PubMed1975

[Lupus erythematosus visceralis in old age].

In the world literature there are only a few reports about systemic lupus erythematosus in older and old patients (from 60 years upwards). In our group of 53 patients with definitive SLE we saw 4 patients (7.5 per cent) with first symptoms after the age of sixty. Specific organic symptoms do not differ from younger patients. But in the course of the disease we can often see pleuropulmonal ans rarely organic kidney symptoms. General symptoms (fatique, languidness, loss of appetite, temperature) are the same as in younger patients. In the 4 patients all laboratory and serological results were very active. Therapy can depress the activity; remissions are rare. In older and old patients SLE should be consideral in the differential diagnosis if the degree of activity is not so clear. In all suspicions we must investigate all important phenomena, especially LE-cells and antinuclear antibodies.

Adult↗
PubMed1976

[Protein-analytical studies on the serum and urine of patients with visceral lupus erythematosus].

Serum and urine of patients with ascertained lupus erythematodes visceralis were separated by means of the polyacrylamide gel pore gradient electrophoresis on the same experimental conditions. Deviations from the normal electropherogram were found in the serum within the gamma-globulins. For the demonstration of antinuclear factors the loose body test, the LE-cell test as well as the immunofluorescence test were carried out. In the uropherogram a highly molecular protein band could be proved which did not appear in the serum. In this fraction an immune complex which developed in the area of the kidney might be in question. It is referred to the possibility of the use of the PAA-polyacrylamide gel electrophoresis for the early recognition of renal changes.

Antibodies, Antinuclear↗
PubMedDate not supplied

[Diagnostic difficulties in a case of visceral lupus with disseminated neurological signs].

The reported case was observed in a patient aged 54 years with disseminated lesions in the central nervous system. The history of the disease covered 18 years with periods of exacerbations and remissions. Seven years after the appearance of neurological signs involvement of the internal organs and skin was noted. In the serum antinuclear antibodies, LE cells and high gamma globulin level were demonstrated. The course of the disease and the observed immunological changes justify the diagnosis of the lupoid sclerosis form of visceral lupus erythematosus.

Antibodies, Antinuclear↗
PubMed1984

[Multiple sclerosis and lupus].

At 20 year-old a patient developed a paraplegia which regressed within several months, suggestive of an acute myelitis. Subsequently, several episodes of spastic paraplegia, posterior tracts lesions and retrobulbar optic neuritis, a transient cerebellar syndrome, modifications in cerebrospinal fluid (pleiocytosis, hypergammaglobulin levels, elevated Delpech's ratio) suggested multiple sclerosis. When aged 62 years, the patient developed articular lesions, Raynaud's phenomenon, and buccal ulcers attributed to lupus. LE cells, native DNA anti-antibodies, anti-Sm auto antibodies on immunofluorescence were present. The possibility of a collagen disease, expressed initially and for a long period in an exclusively neurological disorder is discussed. It was considered, however, to be more likely two distinct affections. This association has been reported very rarely, even though two immunity-mediated inflammatory affections are involved.

Adult↗
PubMed1980

Circumscribed scleroderma with immunologic evidence of systemic lupus erythematosus.

An 8-year-old girl initially manifested clinical and histopathologic findings of circumscribed scleroderma. She had both linear and plaque lesions. Although she was free of constitutional symptoms, laboratory evaluation revealed substantial evidence of systemic lupus erythematosus (SLE). This included a positive anti-nuclear antibody test (rim pattern), positive LE cell preparation test, elevated anti-double-stranded DNA activity, positive lupus band test (IgM and C3) in involved and uninvolved skin, and renal biopsy findings consistent with SLE. Sclerodermatous change as an initial sign of SLE is rare. We review previous reports of an association of circumscribed scleroderma, especially the linear form, with serologic evidence of systemic autoimmune disease.

Antibodies, Antinuclear↗
PubMed1981

[Immunological diagnosis of collagen diseases and rheumatoid arthritis (author's transl)].

Most helpful immunological tests for diagnosis of connectivities are revised. Among them, antinuclear antibodies (AAN) are the most important. They are usually detected by immunofluorescence and show different patterns, being the peripheral one the most specific of SLE and it is related with antibodies anti-DNA which can make immunocomplexes DNA-anti-DNA and be the cause of lupic nephropathy. The "nucleolar" and "speckled" patterns are more frequent in scleroderma. The LE cells have less value than AAN. The rheumatoid factor is a IgM antibody anti-IgG, but also can be of IgG or IgA class. It is uncommon in the juvenile rheumatoid arthritis of systemic onset and more frequent in oligoarticular adult type. May became positive during disease evolution. Other factors as immunoglobulin of complement usually are increased, except when the activation of complement take place. Acute phase reactants are helpful just for evaluating the importance of inflammation, they have not diagnosis value and commonly do not exceed the value of erythrocyte sedimentation rate.

Antibodies, Antinuclear↗
PubMed1983

Lupus erythematosus panniculitis.

A young woman had a history of subcutaneous nodules, migratory arthralgia, myalgia, and lymphadenopathy. Histopathologic characteristics of the nodular subcutaneous lesion showed a massive lobular panniculitis characteristic of lupus erythematosus (LE) panniculitis. The diagnosis of LE panniculitis was established by demonstration of a positive linear basement membrane zone stain of the involved skin by direct immunofluorescence microscopy. Initial serologic findings showed negative antinuclear antibody (ANA) and anti-DNA antibody test results and a negative LE cell preparation. Subsequently, a positive ANA test result developed. The test result for extractable nuclear antigen was strongly positive, although initial test results for antibodies to ribonucleoprotein and Sm antigens were negative.

Adult↗
PubMed1984

Diffuse fasciitis with hypergammaglobulinemia and eosinophilia: a new syndrome?

Two male patients (53 and 19 years old) have been seen with a diffuse scleroderma-like illness with firm taut skin bound down to underlying structures (sparing the face in both patients and the hands and feet in one). Flexion contractures of elbows and knees and limitation of abduction at the shoulders developed in a few weeks after onset. Raynaud's phenomenon was absent and thorough evaluation failed to reveal any evidence of myositis or the visceral manifestations of systemic sclerosis. There was no loss of skin appendages. Both had circulating eosinophils (12% and 37%) without drug ingestion or parasitic infestation. Both had elevated sedimentation rates and hypergammaglobulinemia (in one, IgG = 4.1 g.%). Serologic tests for syphilis, rheumatoid factor, LE cells, antinuclear antibodies, complement and cryoglobulins were negative or normal in both patients. Bone marrow examination revealed plasmacytosis and eosinophilia. Biopsies revealed striking thickening of the fascia between the subcutis and muscle. Within the thickened connective tissue there was intense infiltration with plasma cells and lymphocytes, at times in follicles, both about and separate from vessels; eosinophils were absent. Skin biopsies revealed no changes of scleroderma; and muscle biopsies, no evidence of myositis. Prednisone therapy over 15 months in decreasing dosage, using laboratory parameters, induced in the first patient a full remission which has persisted for 5 years without therapy. In the second (more severely involved) patient prednisone therapy has reversed laboratory abnormalities; but clinical improvement to date has been minimal. The pathogenesis of this diffuse fasciitis is obscure, although unusual physical exertion antedated the onset of illness in each case.

Adolescent↗
PubMed1981

Clinical significance of antinuclear antibodies in systemic lupus erythematosus.

Sera from 70 patients with systemic lupus erythematosus were studied for antinuclear antibodies and its clinical significance. Indirect immunofluorescence technique with mouse liver tissue and Crithidia luciliae flagella as substrates was used to detect antinuclear antibodies (FANA) and antibodies to double-stranded DNA (anti-ds DNA), respectively. Double immunodiffusion method identical with reference sera was used to detect antibodies to extractable nuclear antigens (anti-ENA). The prevalence of fluorescence antinuclear antibodies, antibodies to ds DNA and antibodies to ENA were 95.7%, 52.9% and 58.6%, respectively. Patients with homogeneous pattern of FANA presented clinically with high incidence of LE cell phenomenon. Patients with anti-ds DNA antibodies presented clinically with high incidences of serositis, profuse proteinuria, low serum C3 and high clinical activity. Patients with anti-RNP antibodies presented with high incidence of Raynaud's phenomenon and low incidence of anti-ds DNA antibodies. Patients with severe renal involvement showed a good correlation with high titer of anti-ds DNA antibodies and low serum C3. Therefore, these two factors together are helpful to predict the severity of renal involvement at the time of serum sample was obtained.

Antibodies, Antinuclear↗
PubMedDate not supplied

Systemic lupus erythematosus and thrombotic thrombocytopenic purpura. A case report and review of relationship.

Thrombotic thrombocytopenic purpura (TTP) and systemic lupus erythematosus (SLE) are both multisystem diseases and the latter can manifest hemopoietic abnormalities that may mimic TTP. This has led to diagnostic confusion and reports of the 2 diseases occurring in a single patient. We describe a 15-yr-old girl who presented at age 12 with purpura, fever, headaches, changes in conscious state, thrombocytopenia and microangiopathic hemolytic anemia and who was diagnosed despite the absence of renal disease, as having TTP. ANA and LE cells were negative then, and again 1 yr later. Three yr later she presented with the nephrotic syndrome with a positive ANA (1:100), elevated DNA antibody (79 U/ml, normal less than 25) and a circulating anticoagulant. Renal biopsy confirmed the presence of lupus nephritis. The association is discussed.

Child↗
PubMed1982

[Clinical experience in systemic lupus erythematosus (author's transl)].

Thirty-four cases of SLE treated during the past seven years (1974-1981) in Taipei Municipal Jen-Ai Hospital are reported and analyzed. Diagnosis of SLE was based on ARA preliminary criteria and Hahn's preliminary criteria. There were 32 females (94.2%) and 2 males (5.8%). The mean age at diagnosis was 28.5 years (range 14-51). Clinical manifestations were as follows: facial erythema 24 cases (70.6%), Raynaud's phenomenon 4 cases (11.4%), oral or nasopharyngeal ulceration 7 cases (20.6%), arthritis without deformity 22 cases (64.7%), proteinuria 21 cases (61.8%), pleural or pericardial effusions 13 cases (38.2%), psychosis or convulsions 9 cases (26.5%), hematological abnormalities 25 cases (73.5%). Laboratory findings were as follows: positive ANA test 33/34 (97.0%), hypocomplementemia 10/13 (76.9%), direct Coombs' test 4/18 (22.2%), indirect Coombs' test 1/13 (7.6%), LE cell 19/34 (55.9%), RA Latex 7/17 (41.7%), polyclonal gammopathy 15/17 (88.2%), anemia 25/34 (73.5%), leukopenia 12/34 (35.3%), thrombocytopenia 10/34 (29.4%). Three cases were complicated by herpes zoster, one by hyperthyroidism, and one by autoimmune thyroiditis. Ten cases died, including 4 renal failure, 2 heart failure, 2 cases of committed suicide and 1 case of CNS involvement.

Adolescent↗
PubMed1982

The lupus band test as a measure of disease activity in systemic lupus erythematosus.

One hundred twenty-seven biopsy specimens from clinically normal light-protected skin of 88 patients with active and inactive lupus erythematosus (LE) were examined for deposits of IgG, IgM, IgA, and C3 at the dermal-epidermal junction (DEJ). Deposits were found in 91% of those with active disease and in 33% of those with inactive disease. The finding of such deposits reflected active disease just as did a decrease in serum C3 and C4 levels, elevated anti-double-stranded DNA, the presence of LE cells, lymphopenia, and an elevation of the ESR. The presence or absence of deposits in repeated biopsy specimens indicated changing disease activity, as estimated clinically, just as did changes in the other variables mentioned. Neither immunoreactants in skin nor any other laboratory abnormality reflected renal disease or other type of organ involvement. Deposits of IgG were not more commonly found in patients with renal disease.

Complement C3↗
PubMedDate not supplied

Changing patterns in the clinical features and prognosis of systemic lupus erythematosus - a Japanese experience.

The changing patterns of clinical features and prognosis of 307 Japanese patients with systemic lupus erythematosus (SLE) were studied. SLE patients with Raynaud's phenomenon, alopecia and oral ulceration increased significantly in Group B (1970-1979) compared to Group A (1955-1969). It was noted that SLE patients with LE cells were significantly fewer in Group B and in Group A. The prognosis of Group B was significantly improved over that of Group A. There was a significant decline in deaths caused by uremia and central nervous system lupus in Group B.

Female↗
PubMed1981

[Wegener's granulomatosis with pronounced kidney involvement].

It is reported on the course of histologically ascertained Wegeners' granulomatosis in 4 patients. After initial affection of the upper respiratory tract in form of necrotizing and granulomatous inflammations in all cases a generalisation of the disease with above all early and severe participation of the kidneys developed. Maximum acceleration of sedimentation, C-reactive protein, anaemia, leucocytosis, eosinophilia, thrombocytosis, enlargement of the number of alpha 2-globulins, increase of creatinine, proteinuria, erythrocyturia and leucocyturia are the most frequent pathological laboratory findings, whereas LE-cells, ANF, rheumatoid factor and decrease of the complement never could be proved. By reason of a pathogenic immunoreaction a combined glucocorticoid therapy and immunosuppressive therapy with prednisolone and cyclophosphamide may favourably influence the course of the disease which is otherwise prognostically infaust.

Adolescent↗
PubMedDate not supplied

A systemic lupus erythematosus-like syndrome induced by procainamide.

A syndrome that resembles Systemic Lupus Erythematosus both clinically and on laboratory studies may be induced by procainamide. It is associated with multisystemic involvement, positive Antinuclear Antibody titers (ANA), and positive LE cell preparations. In patients on procainamide therapy the syndrome must be differentiated from osteoarthritis, myocardial infarction, and pulmonary embolism. When procainamide is discontinued, this Lupus-like syndrome is usually reversible. Other drugs, including hydralazine and isoniazid have also been implicated in provoking this Lupus-like syndrome.

Aged↗
PubMed1977

Laboratory procedures used in the diagnosis of systemic lupus erythematosus: a review.

The literature concerning the laboratory procedures presently available to aid in the diagnosis of systemic lupus erythematosus (SLE) was reviewed to determine which of these techniques could be most valuable in the detection and management of SLE patients. The LE cell test, once the laboratory basis for SLE diagnosis, was concluded to be insensitive, non-specific, and did not correspond to clinical activity of the patient. A second procedure, antinuclear-antibody detection, although very sensitive, was not specific for SLE; therefore, its value is limited for use as a screening technique to rule out SLE. The Farr anti-DNA precipitate immunoassay, used for the measurement of antibodies to DNA, was sensitive and specific, and also correlated well with the clinical condition of the patient. Therefore, the Farr binding assay is recommended as the laboratory procedure of choice since it is useful in monitoring disease activity and may contribute to earlier diagnosis and more precise management of SLE patients.

Antibodies, Antinuclear↗
PubMedDate not supplied

The clinical progression of apparent juvenile rheumatoid arthritis to systemic lupus erythematosus.

Ten patients with juvenile rheumatoid arthritis (JRA) developed clinical manifestations of systemic lupus erythematosus (SLE) in 2 1/2 to 21 yr. At onset there was little to distinguish these patients from other children with JRA. Chronic arthritis developed in all and was not different from that seen in JRA. Onset of SLE followed a flare of arthritis in 8 patients, development of serositis in 5, and fever and rash in 5. LE cells and elevated DNA-binding were found in all patients, and glomerulonephritis was demonstrated in the 6 patients on whom renal biopsies were done. This group of patients may represent an important diagnostic subset of children with JRA or SLE.

Adolescent↗