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AB0107 Multiple fibrosis: two cases of successful treatment
  1. NG Guseva,
  2. MN Starovoitova,
  3. TA Nevskaya
  1. Department of Systemic Sclerosis, Institute of Rheumatology of RAMS, Moscow, Russia

Abstract

Objectives Multiple fibrosis (MF) is a slowly progressive disease of unknown cause characterised by localised connective tissue accumulation with the formation of multiple dense sclerotic foci leading to the internal organ dysfunction. It?s a rare and often fatal disease resulting in the visceral organ insufficiency. The most common localizations of the fibrotic lesions are retroperitoneal, mediastinal and lung fibrosis, pseudotumor of pancreas, sclerosing cholangitis. A disease process is usually difficult to stem and often impossible to reverse. There is no specific treatment for MF.

Results We report two cases of successful MF treatment with the combination of D-penicillamine (DPA) and steroids (S). A. 36 yr old male presented with 4 yr history of ?cancer like? symptoms: progressive fatigue, weight loss, subfebrile fiver, accelerating ESR (40 mm/h), pulmonary (dry cough, dyspnoea, atelectasis of the left lower lobe of the lung by X ray and dense ?tumour? 60 mm. in diameter in the core part of the left lung with a massive ?infiltration? spreading along the lower pulmonary vein by thoracotomy) and eye (loss of sight and right eye pain, unilateral scleral congestion) manifestations. With suspicion on the lung and eye cancers the total left pulmonectomy and right eye enucleation were carried out without any state improvement. Two yr later fasciitis, hepatosplenomegaly and new round foci in the right lung (15 mm) and pancreas (27 × 35 mm) were found. MF was confirmed by histopathological finding and the combined therapy with DPA 1000 mg/d and oral steroids 30 mg/d (decreasing to 15 mg daily after 0.5 year and remaining the same for 1.5 year) was prescribed. Over the subsequent 4 months the general condition of the patient improved, the temperature subsided, fasciitis and abdomenalgia disappeared completely, the pt gained 7 kg of weight, ESR diminished to normal levels. In a year we revealed obvious decreasing of the liver and spleen sizes and complete disappearance of the foci in the right lung and pancreas. During a 9 yr follow up we did not find any fibrotic foci in tissues, all laboratory tests are within normal limits. The pt receives daily maintenance dose of 125 mg DPA and 7.5 mg S. No side effects of the drugs have been noticed.

The second patient, a 39 yr old female with Ormond?s disease (retroperitoneal fibrosis which led to squeeze the ureters and bilateral hydronephrosis) had also been operated on – left nephrectomy – before a correct diagnosis was made. The administration of DPA 500 mg/d and S 20 mg/d check the progression of the disease during two years.

Conclusion Our results support data on successful use of the combined therapy with DPA and S in MF. Such drug treatment allowes to get a reverse development of the fibrotic lesions and to maintain a long stable remission of the disease.

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