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[Study on Kveim's test. Positive tests in agammaglobulinemia. Comparison with TTL in presence of Kveim's antigen].

1. -Kveim's test (KT) was carried out on 104 patients for whom the diagnostic of sarcoidosis was suspectid. The positive results of KT correlate with other clinical, radiological and histological criteria. 2. - Two patients with idiopathic agammaglobulinemia (AGG) had positive KT. In two other cases of AGG, KT was negative. Two hypothesis are discussed: a) the association of AGG and infraclinical sarcoidosis favorized by the immunological deficit; b) false positive reactions, which raise the question of the specificity of KT. 3. - Lymphocyte transformation test in the presence of Kveim's antigen (LLT-K) was carried out simultaneously with KT 46 times. Among 30 positive KT only 20 LLT-K were positive. Among 16 negative KT 3LLT-K were positive. LLT-K was the only test carried out on 47 subjects. It was negative among 19 healthy control subjects, 19 allergic subjects and 7 patients suspected of sarcoidosis. LTT-K was positive among 3 patients suspected of sarcoidosis and 1 patient with Hodgkin's disease. 4.- LLT-K like other in vitro tests, presents some advantages over the in vivo tests, in particular the rapidity of response. We found that LTT-K was less often positive when compared with KT. The question of specificity is also raised for LTT-K.

Adolescent

The Edinburgh spleen: source of a validated Kveim-Siltzbach test material.

Although it is true that some Kveim-Siltzbach test suspension may for reasons unknown behave in a totally nonspecific way and so be useless in the confirmation of active sarcoidosis, the experience with the Edinburgh spleen has shown that it is also true that a preparation can be made which acts specifically in the sarcoid context and fulfils all the Siltzbach criteria. The active principle probably resides in the membrane components of sarcoid tissue cells.

Antigens

Acute phase of sarcoidosis with splenomegaly and hypercalcemia. Description of a case, including a report about splenectomy and the preparation and testing of a Kveim antigen from the spleen.

A young man had a mild, slightly progressive pulmonary sarcoidosis. After 4 years he developed an acute disease with splenomegaly, anemia, marked elevation of ESR, hypercalcemia and mild renal insufficiency. The anemia and ESR elevation disappeared after splenectomy, whereas the hypercalcemia still needs corticosteroid treatment. Attempts to withdraw this treatment resulted in recurrence of the hypercalcemia, but no other abnormalities. In contrast to other organs examined, the sarcoid tissue in the spleen revealed necrosis formation, consistent with a recent process. A Kveim antigen preparation from the spleen was less potent than antigen from mediastinal lymph nodes. It is suggested that the acute phase of the disease involved mainly the spleen. Speculations about the possible role of infectious agent(s) are put forward.

Acute Disease

Sarcoid granulomas in CBA/J mice. Histologic response after inoculation with sarcoid and nonsarcoid tissue homogenates.

Homogenates of lymph node and skin biopsy specimens and lymphocyte suspensions from patients with sarcoidosis and control subjects were injected into the footpads of CBA/J mice; histologic response was examined at intervals of 15 to 360 days. Kveim tests and complete autopsies were performed on animals of each group. Granulomas or equivocal granulomas were observed in 14.8% of biopsy specimens from animals that received sarcoid tissue homogenates and in 14.8% of control animals. No granulomas were observed after injection of lymphocytes from patients with sarcoidosis or from control subjects. Kveim tests were negative in all animals and autopsies were unremarkable. These studies fail to confirm previous reports of a "transmissible agent" in sarcoidosis and demonstrate that by the use of these techniques, such an agent is not invariably present in sarcoid granulomas.

Animals

[Experimental findings after application of Kveim antigen. III. Sensitization of mice with living and killed strains of Mycobacterium avium (author's transl)].

Mice were infected with Mycobacterium avium (serotype I and serotype II). The application of kveim antigen or two kinds of lipid fractions of Mycobacterium avium (serotype I or II) is followed in all cases by typical tissue reactions resembling positive kveim tests. Control animals which only received kveim antigen or lipid fractions of mycobacterium I showed in all cases negative kveim reactions. Sensibilization of mice with killed kinds of Mycobacterium avium I or II leads after application of kveim antigen or lipid fractions (outside and inside lipids of Mycobacterium avium I and II) only in few cases to positive kveim reactions.

Animals

Primary intraoral sarcoidosis.

A case of sarcoidosis manifesting primarily in the oral cavity has been documented and clinicopathological findings are discussed. The diagnosis of sarcoidosis can nearly always be established by an assessment of the clinical picture in combination with the histologic features of an adequate biopsy specimen. The Kveim test is a useful tool in clinical diagnosis, but is not always positive in patients with sarcoidosis. The clinician should also be aware that false positives can occur. Scalene node biopsy can also be a useful diagnostic adjunct. The current case emphasizes that biopsy of an intraoral lesion, scalene node biopsy, and Kveim testing are useful in establishing a diagnosis of sarcoidosis.

Diagnosis, Differential

Ichthyosiform sarcoidosis.

A 31-year-old black woman had acquired ichthyosis. Histologic examination of her skin revealed a sarcoidal reaction in the dermis. The diagnosis of sarcoidosis was supported by negative tuberculin (intermediate strength purified protein derivative) and Candida albicans extract intradermal skin tests; by a positive Kveim test; and by roentgenographic evidence of bilateral hilar adenopathy, paratracheal node enlargement, and diffuse pulmonary parenchymal changes. Sarcoidosis must be considered when acquired ichthyosis develops in a patient.

Adult

[Experimental findings after application of Kveim antigen. II. Sensibilization of mice and guinea pigs by Mycobacterium avium (serotype I) (author's transl)].

Mice and guinea pigs were sensibilisized by intraperitoneal injection of Mycobacterium avium (serotype I). Three and five weeks later (guinea pigs) and four weeks later (mice) the animals received kveim antigen into the footpads (guinea pigs) and into the perianal fat tissue (mice). Three and five weeks later (guinea pigs) and four weeks later (mice) the exstirpated material was investigated by light microscopy and enzyme histochemistry. Guinea pigs showed three and five weeks after injection of kveim antigen a lot of questionable positive and positive kveim tests and mice a high percentage of granulomatous changes, resembling a positive kveim reaction. With histochemical methods leucinaminopeptidase is a valuable diagnostic aid for detection of granulomatous changes in mice but not so good in guinea pigs.

Acid Phosphatase

[Experimental findings after application of Kveim antigen. I. Sensibilization of guinea pigs by mycobacterium avium (author's transl)].

Guinea pigs were injected intraperitoneally with different types of mycobacterium avium (serotypes 1, 2 and 3). 4 weeks later followed the injection of Kveim antigen into the foodpats. Beside a high rate of non-specific foreign body reactions in all groups, histological pictures resembling a positive Kveim test were found only in the group of animals which received Mycobacterium avium serotype 1.

Animals