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Gamma-1-Schwerkettenkrankheit mit Immunvaskulitis und rheumatoider Arthritis

Gamma-1 heavy-chain disease with vasculitis and rheumatoid arthritis

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We are reporting about a case of gamma heavy-chain disease (Franklin's disease) with immunovasculitis and rheumatoid arthritis. The diagnosis was confirmed by the results of immunoelectrophoresis of the patient's serum and also by evidence of stimulated lymphocytes without light chain, but having gamma heavy-chain surface proteins.

The immunofixation of the serum showed two protein bands of gamma heavy-chains with different loads. These results are confirmed by a two dimensional electrophoresis and isoelectric focussing of the serum proteins. The pathologic protein consists of at least two different heavy-chain proteins (mol wt 40,000 and 80,000) with isoelectric points between pH 5.5 and 7.3. In the urine of the patient pathological gamma heavy chain-protein was found only in a very low concentration.

The predominant clinical symptom of the patient was a necrotizing vasculitis which became a therapeutical problem. In the immunofluorescence examination of the skin biopsy specimens, immunoglobulins and C3-complement could be detected in the stratum papillare. This fact would be compatible with the development of antibodies or immune complexes against deposited heavy-chain proteins. The arthropathy and the positive rheumatoid factor could similarly be explained by an immune complex mechanism.

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Herrn Univ.-Prof. Dr. Braunsteiner gewidmet

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Stühlinger, W., Berek, K., Lapin, A. et al. Gamma-1-Schwerkettenkrankheit mit Immunvaskulitis und rheumatoider Arthritis. Klin Wochenschr 65, 359–368 (1987). https://doi.org/10.1007/BF01745573

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Key words

  • Heavy-chain diseases
  • Gamma heavy-chain disease
  • Rheumatoid arthritis
  • Immunovasculitis