Case Report

Surgery for Multicentric Reticulohistiocytosis Associated with Arthritis Mutilans: A Case Report with 26-Years’ Follow-up

Hiroaki Kanazawa1*, Akira Hara1, Yuichiro Maruyama1 and Kazuo Kaneko2
1Department of Orthopaedic Surgery, Juntendo University Urayasu Hospital, Japan
2Department of Orthopaedic Surgery, Juntendo University, Japan


*Corresponding author: Hiroaki Kanazawa, Department of Orthopaedic Surgery, Juntendo University Urayasu Hospital, 2-1-1 Tomioka, Urayasu-shi, Chiba 279-0021, Japan


Published: 01 Oct, 2018
Cite this article as: Kanazawa H, Hara A, Maruyama Y, Kaneko K. Surgery for Multicentric Reticulohistiocytosis Associated with Arthritis Mutilans: A Case Report with 26-Years’ Follow-up. Clin Surg. 2018; 3: 2133.

Abstract

We describe a case of Multicentric Reticulohistiocytosis (MR), followed-up for 26 years after diagnosis, which required numerous surgeries involving revision. A 36-year-old woman visited our institution because of distal interphalangeal joint (DIPJ) pain, deformity of the bilateral little fingers, and papulo-nodular cutaneous lesions on both hands. Between 1995 to 2003 arthrodesis of the bilateral thumb, index, middle, ring and little DIPJs were essential. After that, several re-surgeries were needed because of backout of temporary K-wires at the time of follow-up. Furthermore, we performed arthroplasty of the bilateral index, middle, ring proximal interphalangeal (PIP), left thumb metacarpophalangeal joints (MPJs), and right knee joint in 2007 to 2014. Similar, left total knee arthroplasty (TKA) and bilateral total hip arthroplasty (THA) were performed in 2013 to 2016. Postoperative four years after right TKA, aseptic loosening of the tibia and patellar component was gradually recognized. Therefore, we performed revision TKA using long-stemmed augments on the tibial component. The patellar components were removed and we performed patelloplasty only because the height of the remaining patella was thin. The patient was able to walk without a t-cane one year postoperatively. During consecutive follow-up, we experienced some of complications such as the backout of temporary K-wires or nonunion and bone absorption after arthrodesis, and experienced early aseptic loosening after arthroplasty. Therefore, additional surgeries included reinternal fixation or revision arthroplasty were necessary. Postoperatively, surgeons must carefully consider specific bone absorption around the prosthesis or joint in this rare disease.
Keywords: Multicentric reticulohistiocytosis; Surgical treatment; Arthritis Mutilans


Introduction

Multicentric Reticulohistiocytosis (MR) is a rare disease characterized by the proliferation of histiocytes and giant cells in the synovial tissue and the skin. Articular manifestations in MR are similar to those of rheumatoid arthritis (RA): asymmetric articular involvement, marginal erosions, and occasionally severe destructive arthritis. MR is distinguished from erosive osteoarthritis by the lack of osteophytes, ankylosis, periarticular osteoporosis and the presence of soft tissue nodules. Although the mechanism of this synovial proliferation is still unknown, in this case, destructive changes in the finger interphalangeal joints (IPJs), bilateral knee, and hip joint progressed over the years and resulted in severe progressive erosive arthritis. To the best of our knowledge, there are no reports describing long-term survival and clinical results regarding surgical treatment involving revision surgery for this rare disease with mutilans arthritis. We report hereinafter a case of a patient who underwent arthrodesis or arthroplasty of the IPJs, primary total knee arthroplasty (TKA), total hip arthroplasty (THA), and revision TKA over 26-years’follow-up.


Case Presentation

The patient is a 64-year-old woman with involvement of PIPJ and DIPJ of the hand, knee and hip joint. In 1990, (at the age of 36) the patient visited our hospital dermatology department because papulo-nodular cutaneous lesions had appeared on both hands, and received treatment with steroid ointment. Two years later she was diagnosed as MR after biopsies of the subcutaneous nodule. In December, 1993, the patient visited our department because of DIPJ pain and deformity of the bilateral little fingers as well as morning stiffness. On the first examination, radiographs showed bone resorption with marginal erosions similar to those of RA, however there was no periarticular osteoporosis. Next year, the patient also complained of bilateral gonalgia of the right predominance. Laboratory findings showed that the rheumatoid factor was positive (RF 79). Serum C-reactive protein, erythrocyte sedimentation rate, and complement levels were all within normal ranges. In 1995 to 1997, we performed arthrodesis of the left index, the right middle; ring DIPJs using kirschner and soft wires because of bone destruction and subluxation. Subsequently, the patient underwent arthrodesis of the left thumb, middle, ring and little DIPJs because of continuous pain. In 2003, we performed arthrodesis of the right thumb, index and little DIPJs because of instability and weakness of the fingers. Next year, therapy was changed to methotrexate (4 mg/week) and Betamethasone (1 mg/day) because of progressive gonalgia, and then the patient underwent synovectomy of bilateral knee joints. In 2007 to 2008, she underwent arthroplasty using a Swanson flexible silicone implant of the bilateral index PIPJs. Postoperatively, four years after arthroscopic synovectomy, right knee arthralgia recurred, and radiographs revealed bone destructive changes. Therefore, therapy was changed tocilizumab for six months, but the patient’s arthralgia did not improve. The preoperative knee and function scores according to the Knee Society Score were 45 and 35 points, respectively. Preoperative examination revealed destruction of the right knee and middle PIPJs by plain radiographs and intraosseous geodes using Computer Tomography (CT) (Figure 1 and 2). Magnetic Resonance Imaging (MRI) of the right knee showed destructive changes with well circumscribed marginal erosions; there were bulky masses with a relatively high intensity on T1-weighted fat suppression images in the joint space and extending to the suprapatellar bursa (Figure 3). In 2009, we finally performed cemented arthroplasty of the knee and middle PIPJs (Avanta, Small Bone Innovations, and USA) (Figure 4). The intraoperative findings revealed that articular cartilage had disappeared and the articular surface was covered to the synovium. Bone defect of the posteromedial tibial condyle was recognized. Therefore, we operated using the PFC sigma knee prosthesis. (A femur component was used with cruciate substituting (CS) and a femoral stem extension. The tibia component used was modular plus hemiwedge metallic augments, stem extension (Depuy, Warsaw, Indiana, USA) Patellae were resurfaced with use of a domed, 3-pegged, all-polyethylene component. Subsequent histopathologic examination of the tissue specimen revealed a proliferation of histiocytes and synoviocytes with multinucleated giant cells seen in synovium of the knee (Figure 5). Similarly, contralateral TKA was performed. Intraoperatively, the preserved peripheral shell of the postero medial site of the tibia and femoral medial condyle were observed. Therefore, the bone defect was filled using an autograft harvested from the osteomized bone. A femur component was used for CS with a femoral stem extension. The tibia component used was modular plus hemi wedge with an extension stem (Depuy, Warsaw, Indiana, USA) (Figure 6). Also, bilateral hip arthritis was progressive, and we performed THA (SL-PLUS MIA system, oxinium head diameter: Smith & Nephew) of the left side in 2015 and of the right side in 2016. Postoperatively, four years after the right TKA, aseptic loosening combined with varus deformity of the tibia and loosening of the patellar component were gradually recognized and progressed (Figure 7). Therefore, we performed revision TKA using long-stemmed augments on the tibial component (Tibia MBT revision tray size plus stem wedge, stem 10 mm to 115 mm, Rotating Platform insert: Depuy, Warsaw, Indiana, USA) and allograft. The patellar component was removed and we performed patelloplasty only, because the height of the remaining patella was thin (about 10 mm). Intraoperatively, loosening of the femur components was not recognized. Chronological listing of surgical procedures and medical treatments in this case are summarized in Table 1 and 2. Although there were some clinical symptoms like crepitation and discomfort while the range of motion of the knee remained to some extent, the patient was able to walk without a t-cane after one year post-surgery (Figure 8). The knee society knee and functional scores were 88 and 70 points, respectively.


Figure 1

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Figure 1
X-ray (A),(B): Anterioposterior and Lateral view of the knee in 2009. (C): Anteroposterior view of the right hand.

Figure 2

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Figure 2
Destruction of the articular surface, and intraosseous geodes were recognized on 3-dimential Computer Tomography (CT) of femur (A) and tibia (B).

Figure 3

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Figure 3
MRI on T1-weighted fat suppression images showed bulky masses extending to the suprapatellar bursa (Axial view).

Figure 4

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Figure 4
Right TKA and right middle finger PIP arthroplasty was performed in March 2009 (A) Anteroposterior (B) Lateral view of the knee (C) anteroposterior view of the hand.

Figure 5

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Figure 5
Histopathological examination (hematoxylin and eosin stain ×100), demonstrating a proliferation of histiocytes and synoviocytes with multinucleated giant cells.

Figure 6

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Figure 6
Anteroposterior and lateral view of the left TKA in 2013.

Figure 7

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Figure 7
Four years after right TKA, aseptic loosening of the tibia and patella components was recognized.

Table 1

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Table 1
Chronological listing of surgical procedures.

Figure 8

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Figure 8
Anteroposterior and lateral view of the right TKA revision in 2016.

Table 2

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Table 2
Chronological listing of medical treatment.

Discussion

To date, the surgical management of MR with arthritis mutilans has not been widely described. To our knowledge, there are no reports describing long-term survival and clinical results regarding surgical treatment involving revision surgery for patients with MR. The disease is characterized by tissue infiltration of lipid laden histiocytes and multinucleated giant cells. Such changes in the bone and synovium can result in destructive arthritis, although the mechanism of arthritis in MR is poorly understood. Nakajima et al. [1] reported that MR cells express IL-1β and platelet-derived growth factor B. Zagala et al. [2] have speculated that osteolysis in MR is caused by cytokines, including IL-1, and chemical mediators, such as prostaglandin E2, that are produced by the histiocytic cells. Roentgenographic findings may show erosive changes very similar to those of RA. As in RA, they tend to be bilaterally symmetric. But significant erosive changes of DIPJs are not common in RA. Circumscribed erosions spread over the articular surface, resulting in widening of joint space, loss of cartilage, and resorption of subchondral bone. These changes are found particularly in IPJs (75% of patients with this disease). Changes may be also evident in other joints of the appendicular skeleton. MR is distinguished from erosive osteoarthritis by the lack of osteophytes, ankylosis, periarticular osteoporosis and the presence of soft tissue nodules. In this case, the patient progressed to destructive arthritis mutilans in our observation period of 26 years. An internal medicine treatment for MR has not yet been established due to the few published reports of this disease. Immunosuppressive or cytotoxic agents or corticosteroids and methotrexate were used in treatment of this disease [3,4]. Recently, anti-TNF-α agents and alendronate have been tried and they appear to offer another effective therapeutic option [5-7]. More recently, tocilizumab was used as monotherapy or in combination with methotrexate or other disease-modifying antirheumatic drugs for the treatment of RA, and it is an effective agent targeting the interleukin-6 cytokine receptor [8]. Therefore, we tried this agent for MR experimentally this time. However, our patient developed severe joint destruction despite aggressive therapy. Thereafter, we decided on surgical treatment. However, surgical treatment seems to have been rarely reported previously in the literature [9,10]. In the case of surgery, we would mention the need to evaluate intraosseous geodes or the bone defect by CT and MRI as well as an X-ray preoperatively [11]. Previous reports do not offer a surgical algorithm; therefore we would like to propose a pitfall with regard to surgical treatment for this disease through our experience. During consecutive follow-up, we experienced complications such as the backout of temporary K-wires or nonunion and bone absorption after arthrodesis of the DIPJs in hand. Furthermore, we experienced early aseptic loosening combined with varus deformity after TKA. Therefore, several additional surgeries were necessary. We take into consideration the risk of postoperative early aseptic loosening and therefore bone fragility must be kept in mind at the time of surgery. There is no available evidence to answer questions regarding the surgical treatment of patients with MR and it is unknown as to whether arthroplasty is an effective treatment. Arthrodesis may be indicated in small joint arthritis of the hand, but we consider that arthrodesis does not deliver satisfactory results functionally for knee or hip joint arthritis. Therefore, arthroplasty is selected, but bone absorption around the prosthesis is a problem.


Conclusion

This rare case should remind orthopaedic surgeons and radiologists to consider MR in the differential diagnosis of progressed to destructive arthritis mutilans. We were able to consecutively follow-up for 26 years after diagnosis and report our surgical experience. We would be pleased if this case presentation helps the surgical management and elucidation of this disease. Careful followup and further investigation of clinical courses will be necessary in the future.


References

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