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[Polymyositis and Jo-1 syndrome]
E Treher1, A Niederhoff, U Gellissen
1Abteilung für Rheumatologie und Klinische Immunologie, Medizinische Universitätsklinik, Freiburg.
Zeitschrift Fur Rheumatologie
|September 1, 1993
Summary
Jo-1 syndrome, characterized by anti-histidyl-tRNA-synthetase antibodies, often presents with interstitial lung disease. Early immunosuppressive therapy, including corticosteroids, can improve outcomes in patients with Jo-1 associated pulmonary fibrosis.
Area of Science:
- Rheumatology
- Pulmonology
- Immunology
Background:
- Polymyositis is diagnosed clinically, but anti-Jo-1 antibodies (anti-histidyl-tRNA-synthetase) are present in 30-40% of cases.
- Jo-1 syndrome encompasses these cases, frequently involving interstitial lung disease and other characteristic symptoms.
Observation:
- The case report details a patient with Jo-1 syndrome and early pulmonary fibrosis.
- Initial treatment with methotrexate and prednisolone showed a positive response.
Findings:
- After one year, the patient's condition worsened, necessitating a change in therapy.
- Treatment was switched to cyclophosphamide, with long-term outcomes pending evaluation.
Implications:
- Early diagnosis of Jo-1 associated interstitial lung disease is crucial for effective treatment.
- Immunosuppressive therapy, particularly corticosteroids, can favorably influence the prognosis of pulmonary fibrosis in Jo-1 syndrome.
- Cyclophosphamide represents a potential therapeutic option for refractory cases, though further evaluation is required.