New Candidate Criteria for Diagnosis of Macrophage Activation Syndrome

NCT01095146 | Unknown

• 1 other identifier: K-PAE-10-284
• Study Type: observational
• Target: 50
• Locations: 1 country
• Sites: 1

Brief Summary

Macrophage activation syndrome(MAS) is a complication of bone marrow suppression, coagulopathy and CNS dysfunction which occurs in rheumatic diseases. Normally the (Hemophagocytic Lympho-Histiocytosis) HLH-2004 criteria is used to diagnose patients with MAS. However this criteria is probably not sensitive and would probably be fulfilled quite late into the disease. Thus there would be an unacceptable delay. Ravelli et al came up with a different set of criteria based on data of patients reported in literature. Systemic onset juvenile idiopathic arthritis (SoJIA) is the most common cause of MAS. MAS in other rheumatic illnesses occurs in the setting on unbridled inflammation. In both SoJIA and uncontrolled rheumatic disease the patient is liable to have high WBC counts and high platelet counts. Bone marrow suppression which is one of the pathognomic features of MAS would be picked up very late if absolute cut off values were utilized. Kelly et al used the same arguments in their review to suggest that in MAS/Reactive hemophagocytic lymphohistiocytosis(ReHLH), the trend of change in laboratory parameters would be more useful than absolute cut offs. Hence the investigators propose new candidate criteria which are based on trends of laboratory parameters and seek to determine their utility in comparison to absolute cut offs of HLH or Ravelli criteria. The investigators also wanted to determine that among the Ravelli criteria and HLH-2004 criteria, which were fulfilled earlier in patients diagnosed as having MAS. Study hypothesis:-Criteria which measure serial trend of laboratory parameters would be fulfilled earlier than absolute cut offs when diagnosing MAS in patients with rheumatic illness.

Conditions

Macrophage Activation Syndrome; Lymphohistiocytosis, Hemophagocytic

Keywords

MAS; Hemophagocytosis; Reactive HLH; Macrophage activation syndrome; Systemic onset juvenile idiopathic arthritis; SoJIA; JIA

Eligibility Criteria

• Sex: all
• Healthy Volunteers: No
• Age Groups: Child (0-17), Adult (18-64), Older Adult (65+)
• Sampling Method: Non-Probability Sample

Study Population

Patients admitted in the Pediatrics, Internal Medicine, Rheumatology/Clinical Immunology departments of hospitals

You may qualify if:

• All the patients diagnosed as having Macrophage Activation Syndrome at death or discharge from 1st Jan 2004 to 1st Jan 2012, in all the centers enrolled as collaborators of the study would be included in the study

You may not qualify if:

• Subjects with a final diagnosis of (Infection associated hemophagocytic syndrome) IAHS
• Subjects with a genetic diagnosis of HLH
• Subjects with a family history (1st degree relative) of a genetic diagnosis of HLH.

Sponsors & Collaborators

• Amrita Institute of Medical Sciences & Research Center: lead

Study Sites (1)

Amrita Institute of Medical Sciences

Kochi, Kerala, 682041, India

Location

Related Publications (3)

• Kelly A, Ramanan AV. Recognition and management of macrophage activation syndrome in juvenile arthritis. Curr Opin Rheumatol. 2007 Sep;19(5):477-81. doi: 10.1097/BOR.0b013e32825a6a79.

  PMID: 17762614 BACKGROUND

• Ravelli A, Magni-Manzoni S, Pistorio A, Besana C, Foti T, Ruperto N, Viola S, Martini A. Preliminary diagnostic guidelines for macrophage activation syndrome complicating systemic juvenile idiopathic arthritis. J Pediatr. 2005 May;146(5):598-604. doi: 10.1016/j.jpeds.2004.12.016.

  PMID: 15870661 BACKGROUND

• Henter JI, Horne A, Arico M, Egeler RM, Filipovich AH, Imashuku S, Ladisch S, McClain K, Webb D, Winiarski J, Janka G. HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer. 2007 Feb;48(2):124-31. doi: 10.1002/pbc.21039.

  PMID: 16937360 BACKGROUND

MeSH Terms

Conditions

Macrophage Activation Syndrome; Lymphohistiocytosis, Hemophagocytic; Arthritis, Juvenile

Condition Hierarchy (Ancestors)

Lymphoproliferative Disorders; Immunoproliferative Disorders; Immune System Diseases; Histiocytosis, Non-Langerhans-Cell; Histiocytosis; Lymphatic Diseases; Hemic and Lymphatic Diseases; Arthritis; Joint Diseases; Musculoskeletal Diseases; Rheumatic Diseases; Connective Tissue Diseases; Skin and Connective Tissue Diseases; Autoimmune Diseases

Study Officials

• Sharath Kumar, MD

  Amrita Institute of Medical Sciences

  PRINCIPAL INVESTIGATOR

Study Design

• Study Type: observational
• Observational Model: CASE ONLY
• Time Perspective: RETROSPECTIVE
• Sponsor Type: OTHER

Study Record Dates

• First Submitted: March 29, 2010
• First Posted: March 30, 2010
• Study Start: March 1, 2010
• Primary Completion: January 1, 2012
• Study Completion: January 1, 2012
• Last Updated: March 30, 2010

Record last verified: 2010-03

Locations

IN (1)