Treatment of Severe Multicentric Reticulohistiocytosis With Upadacitinib

Niaki, Omid Zahedi; Penn, Erin H.; Scott, Deborah A.; Cobos, Gabriela; Vleugels, Ruth Ann; Weinblatt, Michael E.

doi:10.1001/jamadermatol.2021.0996

Cited by 7 publications

(9 citation statements)

References 7 publications

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“…Tofacitinib was reported by Bruscas Izu C. et al to be a possible therapeutical alternative when antirheumatic drugs classically used in MRH, namely prednisone, methotrexate, and etanercept, fail to improve articular and skin manifestations [ 30 ]. Moreover, a severe case of MRH, resistant to first-line therapies, showed a marked improvement in cutaneous lesions and arthritis following treatment with upadacitinib, as reported by Niaki OZ et al [ 31 ]. Although an empiric algorithm for the treatment of MRH exists, a large, single-center case series is needed to clarify its actual incidence, associated systemic disorders, and therapy responses.…”

Section: Discussionmentioning

confidence: 66%

Multicentric Reticulohistiocytosis Associated with an Early Form of Systemic Lupus Erythematosus: A Case Report of a Rare Disease, with Mini Review of the Literature

Mariotti

Corrà

Lemmi

et al. 2022

JCM

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Multicentric reticulohistiocytosis (MRH) is the most frequently described form of reticulohistiocytosis (RH), and it is classified as a class IIb non-Langerhans cell histiocytosis. It has been designated as multicentric, being characterized by multisystemic involvement. In fact, although mainly involving the skin, along with the joints, it is a systemic inflammatory condition potentially involving every internal organ. As MRH-related skin findings can mimic rheumatoid nodules or Gottron papules, the histopathology of the cutaneous lesions is often necessary for the correct diagnosis. Approximately one-third of MRH patients have confirmed concomitant autoimmune disorders. A wide variety of autoimmune disorders associated with the disease have been reported in the literature, suggesting immune dysfunction as a factor in the pathophysiology of MRH. A case of MRH associated with autoimmune manifestation is reported in the context of a mini review of the literature, with a focus on clinical presentation, treatments, and treatment outcomes. Moreover, eight cases of MRH associated with autoimmune diseases are briefly discussed.

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Section: Discussionmentioning

confidence: 66%

Multicentric Reticulohistiocytosis Associated with an Early Form of Systemic Lupus Erythematosus: A Case Report of a Rare Disease, with Mini Review of the Literature

Mariotti

Corrà

Lemmi

et al. 2022

JCM

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“…The initial price of the biologics was relatively high and medical insurance in China could not cover them during these years, which might be the most important reason why these patients did not choose these drugs. However, biologics might be a beneficial choice for MRH patients [ 5 , 18 , 25 – 27 ]. In fact, it seemed that most patients with MRH had a satisfactory response to these medications.…”

Section: Discussionmentioning

confidence: 99%

Clinical characteristics of multicentric reticulohistiocytosis and distinguished features from rheumatoid arthritis: a single-center experience in China

Zou

Lin

et al. 2022

Orphanet J Rare Dis

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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 patients were women. The median age was 46 years (range 33–84 years). Diagnosed by specific pathologic features, all MRH patients exhibited cutaneous involvement. The dorsa of the hands, arms, face and auricle were the most commonly affected areas. Nodules were also located on the legs, scalp, trunk, neck, and even the hypoglossis and buccal mucosa. Ten MRH patients (90.9%) had symmetric polyarthritis. Compared with rheumatoid arthritis (RA) patients, MRH patients were more likely to have distal interphalangeal joint (DIP) involvement (63.6% vs 24.2%, P = 0.017) and less likely to have elbow (36.4% vs 72.7%, P = 0.003), ankle (45.5% vs 93.9%, P < 0.001) and metacarpophalangeal joint (MCP) (36.4% vs 78.8%, P = 0.009) involvement. Positivity for rheumatoid factor (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], in MRH patients was lower than in RA patients. Elevation of the erythrocyte sedimentation rate (ESR) was also less common in MRH patients than in RA patients (36.4% vs 72.7%, P = 0.030). After treatment with median- to large-dose corticosteroids and disease-modifying antirheumatic drugs, 8 patients achieved complete remission and 2 patients partial remission (skin lesions ameliorated, joint lesions not ameliorated). Conclusion Always pathologically diagnosed, MRH is a systemic disease involving RA-like erosive polyarthritis and a specific distribution of skin nodules characterized by "coral beads". More DIP involvement and less elbow, ankle and MCP involvement are seen in MRH than in RA. In addition, less positivity and lower-titer RF, uncommon presence of anti-CCP antibodies and ESR elevation may be helpful to distinguish MRH from RA.

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“…Given that its clinical manifestations are similar to rheumatic conditions, it has been proposed that MRH is an autoimmune or inflammatory disease, so treatments such as the administration of corticosteroids, methotrexate, thalidomide (34), bisphosphonates, and biological anti-inflammatory agents [etanercept, adalimumab (35), infliximab, tofacitinib (36), upadacitinib (37)] have been employed with varying efficacy. Although our patient was sensitive to methotrexate treatment, we had to discontinue it because of the malignancy.…”

Section: Discussionmentioning

confidence: 99%

Case Report: Multicentric Reticulohistiocytosis Associated With Posterior Mediastinal Adenosquamous Carcinoma, Antinuclear Antibody Positivity and Lupus Anticoagulant Positivity

et al. 2022

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Multicentric reticulohistiocytosis (MRH) is a rare systemic disease of non-Langerhans cell histiocytosis. A number of studies in the literature have documented that it can coexist with malignancy or autoimmune disease, making it difficult to determine the most appropriate therapy. Here, we present a case study of MRH associated with posterior mediastinal adenosquamous carcinoma along with antinuclear antibody positivity and lupus anticoagulant positivity. The patient experienced 6 months of clinical benefit after surgical resection and chemoradiotherapy of the mediastinal malignancy. This case adds to the available literature on multicentric reticulohistiocytosis associated with different types of malignancy and provides supplementary clinical data on the coexistence of this syndrome with malignancy and immune system abnormalities. To the best of our knowledge, this is the first case study describing MRH accompanied by posterior mediastinal adenosquamous carcinoma and lupus anticoagulant positivity. The unknown aetiology and polymorphic clinical presentation of MRH warrants further investigation.

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Treatment of Severe Multicentric Reticulohistiocytosis With Upadacitinib

Cited by 7 publications

References 7 publications

Multicentric Reticulohistiocytosis Associated with an Early Form of Systemic Lupus Erythematosus: A Case Report of a Rare Disease, with Mini Review of the Literature

Multicentric Reticulohistiocytosis Associated with an Early Form of Systemic Lupus Erythematosus: A Case Report of a Rare Disease, with Mini Review of the Literature

Clinical characteristics of multicentric reticulohistiocytosis and distinguished features from rheumatoid arthritis: a single-center experience in China

Case Report: Multicentric Reticulohistiocytosis Associated With Posterior Mediastinal Adenosquamous Carcinoma, Antinuclear Antibody Positivity and Lupus Anticoagulant Positivity

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