title: Thalidomide for improving cutaneous and pulmonary sarcoidosis in patients resistant or with contraindications to corticosteroids.
abstract: BACKGROUND
  Limited data report thalidomide improves cutaneous sarcoidosis; no benefit has been reported for pulmonary localization.
  
  
  OBJECTIVE
  To evaluate feasibility and efficacy of prolonged treatment with thalidomide for cutaneous sarcoidosis associated to pulmonary involvement in patients with resistance or contraindications to steroids.
  
  
  METHODS
  Nineteen patients were treated with thalidomide for 24 months starting with 200 mg/d for first 2 weeks, followed by 100 mg/d for 11 weeks and a maintenance dose of 100mg on alternate days for 35 weeks, and a gradual scaling down until therapy interruption. Criteria of efficacy were: skin score, serum ACE levels (s-ACE), chest X-ray (CXR), lung function tests (LFTs), and diffusing lung capacity for CO (DLCO). The skin score was computed as arithmetic sum of seven score parameters (min: 0, max: 28).
  
  
  RESULTS
  Skin score significantly decreased (P<0.001). Lower skin scores occurred after 3 and 6 months (P<0.05). s-ACE levels decreased over time at the third month (P<0.001). CXR assessed by radiological stage significantly improved during the first 6 months (P<0.001). DLCO showed a continuous trend of improvement. Minor side effects that have forced the suspension of the drug were drowsiness/sedation (74%), constipation (68%), and weight gain (53%). Deep vein thrombosis of the lower limbs occurred in one patient (who did not drop out the study). Eight patients (42%) abandoned thalidomide for axonal sensitive peripheral neuropathy (PN) between the ninth and the 24th month of treatment.
  
  
  CONCLUSIONS
  Thalidomide, long-term at mid-low doses, can be considered as an effective therapeutic alternative in chronic sarcoidosis with resistance or contraindications to steroids.
authors.affiliation: Respiratory Pathophysiology Section, Cardiothoracic and Vascular Department, University of Pisa, Pisa, Italy.
authors.forename: P
authors.initials: P
authors.lastname: Fazzi
authors.forename: E
authors.initials: E
authors.lastname: Manni
authors.forename: R
authors.initials: R
authors.lastname: Cristofani
authors.forename: G
authors.initials: G
authors.lastname: Cei
authors.forename: S
authors.initials: S
authors.lastname: Piazza
authors.forename: R
authors.initials: R
authors.lastname: Calabrese
authors.forename: A
authors.initials: A
authors.lastname: Antonelli
authors.forename: G
authors.initials: G
authors.lastname: Siciliano
authors.forename: P
authors.initials: P
authors.lastname: Barachini
authors.forename: A
authors.initials: A
authors.lastname: Carpi
mesh_terms: D000328:Adult; D000368:Aged; D000075202:Contraindications; D004305:Dose-Response Relationship, Drug; D004351:Drug Resistance; D005240:Feasibility Studies; D005260:Female; D005500:Follow-Up Studies; D005938:Glucocorticoids; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D012507:Sarcoidosis / Q000188:drug therapy* / Q000473:pathology; D017565:Sarcoidosis, Pulmonary / Q000188:drug therapy* / Q000473:pathology; D012871:Skin Diseases / Q000188:drug therapy* / Q000473:pathology; D013792:Thalidomide / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use*; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D013792:Thalidomide