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Multicentric reticulohistiocytosis: systemic macrophage disorder
Bailliere'S Clinical Rheumatology
|August 1, 1991
Summary
Multicentric reticulohistiocytosis (MRH) is a rare multisystem inflammatory disease. Early, aggressive treatment is recommended to prevent severe joint and skin damage, though evidence is largely anecdotal.
Area of Science:
- Rheumatology
- Dermatology
- Pathology
Background:
- Multicentric reticulohistiocytosis (MRH) is a rare multisystem disorder.
- Characterized as a dermatoarthritis, it can affect multiple organ systems, including cardiac and skeletal muscle, pleura, and the gastrointestinal tract.
- Synovitis can be destructive, leading to arthritis mutilans, and dermatitis can be disfiguring, especially when the face is involved.
Purpose of the Study:
- To describe the clinical and laboratory features of Multicentric Reticulohistiocytosis (MRH).
- To review previously reported cases and analyze recent literature.
- To note changes in the frequency of MRH manifestations.
Main Methods:
- Review of 33 previously reported cases of MRH.
- Analysis of an additional 33 cases from the medical literature since the prior review.
- Examination of clinical and laboratory features.
Main Results:
- An apparent decline in the frequency of some MRH manifestations was noted.
- Tuberculosis exposure was present in one-third of cases (5% active TB).
- Malignancies were concomitantly reported in 15-28% of cases.
Conclusions:
- The primary cell involved in MRH is the phagocytic tissue histiocyte (macrophage).
- No direct evidence links MRH to a specific organism or neoplasm.
- Early and aggressive treatment is trending to prevent arthropathy and cutaneous sequelae, but recommendations are based on anecdotal reports, requiring cautious clinical approach.