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Abstract Objective To investigate the clinical features of multicentric reticulohistiocytosis (MRH). Methods The clinical manifestations, laboratory examination results and histologic characteristics of eleven patients with MRH were collected and compared with those of 33 patients with rheumatoid arthritis. Results In total, 72.7% of the MRH patien...
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... for RF (36.4% vs 84.6%, P = 0.001) and anti-CCP antibody (9.1% vs 81.8%, P = 0.000), as well as the median RF titer [43.8 (31.7-61.0) vs 175.4 (21.3-940.3), P = 0.021] (Fig. 5), in MRH patients was lower than that in RA patients. Elevation of ESR was less common in MRH patients than in RA patients (36.4% vs 72.7%, P = 0.030) and showed significance. There was no significant difference in age, sex, antinuclear antibody (ANA), C-reactive protein (CRP) or other joint involvement, including knee joints, PIP ...
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... It is more prevalent (80%) in Caucasian females in their fifth to sixth decade of life [1,2]. Male to female ratio of the MRH prevalence is 1:3 [3,4]. Skin-colored papulonodular lesions are typically seen all over the body. ...
... Patients usually demonstrate symptoms and signs of symmetric polyarthritis and papulonodular cutaneous lesions ( Figure 1). Pericardial involvement is a rare manifestation ( Figures 5, 6), and around three cases have been reported in the literature so far [1,3,6]. Our case report is a valuable contribution to the literature, which aids clinicians in contemplating MRH as one of the differentials among patients presenting with pericardial effusion. ...
... Synovial fluid analysis of the involved joints has been studied to be indicative of a non-inflammatory type of effusion in 75% of patients [10]. Around 20-75% of the patients with MRH have positive anti-nuclear, anti-citrullinated peptide, anti-Ro, and anti-SSA antibodies, which can explain the relation between MRH and connective tissue disorders [3,5]. Histologically, multinucleated giant cells and histiocytes are seen with eosinophilic ground glass cytoplasm. ...
Multicentric reticulohistiocytosis (MRH) is a rare, class IIb non-Langerhans cell histiocytosis associated with skin and joint involvement. It is more prevalent (80%) in Caucasian females in their fifth to sixth decade of life. Patients usually demonstrate symptoms and signs of symmetric polyarthritis and papulonodular cutaneous lesions. In addition to skin and joints, multiple organs can be involved, such as the lung (pleural effusion, interstitial fibrosis, hilar lymphadenopathy), heart (pericardial effusion, myocarditis), gastrointestinal system, and urogenital system (genital tract and kidney). Pericardial involvement is a rare manifestation, and around three cases have been reported in the literature so far. Our case report is a valuable contribution to the literature, which aids clinicians in contemplating MRH as one of the differentials among patients presenting with pericardial effusion. We described the characteristics of MRH along with its differentiating features from other autoimmune conditions and management.
... 11 Muscular involvement in the form of proximal muscle weakness and myalgia has also been documented. 12 In up to a third of patients, MRH may co-exist with other autoimmune conditions such as rheumatoid arthritis (RA), Systemic Lupus Erythematosus (SLE), Sjogren's syndrome, scleroderma or hypothyroidism. [13][14][15] Rheumatoid Factor, ANA and anti-CCP are usually negative but in case of overlapping autoimmune diseases antibodies may be positive. ...
... In case of concomitant malignancy, treatment of malignancy may lead to resolution of MRH. 12,19 CONCLUSION MRH is a rare disease of unknown aetiology. We presented a case of a Pakistani man with MRH who presented with typical maculopapular rash and inflammatory arthritis. ...
Multicentric Reticulohistiocytosis is a rare disorder of unknown aetiology which affects skin and joints predominantly. There are no specific laboratory investigations for diagnosis. Diagnosis can be made clinically and on a histopathological basis. There is no consensus on treatment. We report a case from Pakistan with classical presentation who did well on methotrexate and low dose steroids. Prompt diagnosis and early treatment may save from significant disability.
