Simultaneous bilateral total hip arthroplasty for managing hip arthropathy secondary to Dianzani autoimmune lymphoproliferative disease: A case report

INTRODUCTION

Musculoskeletal manifestations of autoimmune diseases are common, with large joints, including the hips, commonly involved in some diseases.^[1,2] Hip joint affection could be part of the disease process or a complication of the medical treatment, such as osteonecrosis of the femoral head (ONFH) secondary to corticosteroid therapy.^[3] Furthermore, in most cases, the disease is presented bilaterally.^[2]

In cases of advanced bilateral hip diseases, such as ONFH or osteoarthritis (OA), necessitating surgical intervention, the management of choice is total hip arthroplasty (THA), which offers excellent functional outcomes and improves quality of life.^[4,5] The surgeon could choose to proceed with simultaneous bilateral THA (where both hips are being replaced at the same session) or to perform sequential THA (where one side is being replaced and the other after a few months), and each approach has its benefits and risks.^[6-8]

Apart from bilateral hip joint affection associated with commonly known autoimmune diseases, such as rheumatoid arthritis,^[1,3] surgeons might face rare conditions, such as Dianzani autoimmune lymphoproliferative disease (DALD).^[9] This disease is a variant of the autoimmune lymphoproliferative syndrome (ALPS) characterized mainly by autoimmunity, lymphadenopathy, and/or splenomegaly; however, various joint affections might be part of the disease’s clinical presentation or could result as a complication from its treatment protocols.^[10]

In patients with bilateral hip disease necessitating THA in conjunction with an autoimmune disease, collaboration between the treating rheumatologist or internist, the anesthesiologist, and the surgical team is paramount. To highlight the importance of such a collaboration and how the proper diagnosis could be reached, we present a case of bilateral ONFH in a female patient diagnosed with a rare DALD, which was successfully treated by simultaneous bilateral cementless THA.

CASE REPORT

This case report was prepared as per the Consortium for Academic and Research Ethics guidelines.^[11]

Patient details and initial assessment

A 34-year-old female presented with bilateral hip pain and limitation of motion, which had deteriorated over the past few years. She reported being diagnosed with an autoimmune disease; throughout the disease course, she has been receiving a variety of immune system-modulating drugs and steroids. Upon contacting her treating rheumatologist (F.F.), she reported that the patient was diagnosed with DALD, a very rare autoimmune disorder characterized by various presentations, including recurrent episodes of thrombocytopenia, neutropenia, and/or autoimmune hemolytic anemia, lymphadenopathy, and/or splenomegaly. The approach to diagnosis and medical treatment for managing DALD is illustrated in a flow diagram [Figure 1].^[12]

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Figure 1:

A flowchart showing the patient’s history from the start of the disease and how she was diagnosed over the years. Dianzani autoimmune lymphoproliferative disease (DALD) depends on the diagnosis of an early onset with: 1) Autoimmunity, which is predominantly hematological, but any other autoimmunity can be displayed. 2) Enlargement of the spleen and/or lymph nodes due to accumulation of polyclonal lymphocytes. 3) Decreased function of the Fas death receptor. Patients lack the peripheral blood expansion of T cells expressing the TCR alpha/TCR beta but not CD4 and CD8 (double-negative T cells), which are present in the typical form of Autoimmune Lymphoproliferative Syndrome. (TNF: tumor necrosis factor, TCR: T-cell receptor, CBC: complete blood count, MCP: metacarpophalangeal joint, PIP: proximal interphalangeal joint).

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Clinical evaluation

Upon presentation to the orthopedic clinic, she was walking with an antalgic gait using a walker, and there were obvious bilateral hand deformities [Figure 2a]. Upon lying on the examination bed, she had apparent bilateral hip flexion deformities, which were later confirmed by performing the Thomas test. There was bilateral tenderness over the hip joint during the local hip examination. The range of motion was limited on both sides, 80° and 70° of flexion, 20° and 10° of abduction, for the right and left hips, respectively. Both hips had 10° of adduction, 10° of external rotation, and 10° of internal rotation. She had bilateral knee flexion deformities of 10° on both sides. Her functional score, as per the Harris Hip Scoring (HHS) system, was 34.5 and 34.2 for the right and left hips, respectively.

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Figure 2:

Clinical and radiological images. (a)Bilateral hand deformity was present clinically and in the plain radiographs. (b)Anteroposterior and lateral plain radiographic views of the hips bilaterally showing the diminished joint space and osteonecrotic patches in the femoral heads (yellow arrows). (c) Magnetic resonance images show the femoral head’s osteonecrosis (red arrows and circles) extending to the femoral neck bilaterally (green arrow).

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Post-operative protocol and outcomes

The patient was transferred to the intensive care unit (ICU) postoperatively for proper monitoring, where she spent 2 days, and then she was transferred to the inpatient ward. Her in-hospital post-operative course was unremarkable. The immediate post-operative plain radiographs showed proper implant positioning and equal leg length [Figure 3a]. The patient was mobilized on the 3^rd post-operative day under the supervision of the physiotherapy team. Antibiotic prophylaxis was given in the form of a first-generation cephalosporin, 1 g IV every 8 h starting 30 min before surgery till the first 24 h, then the patient was given an extended antibiotic prophylaxis in the form of 500 mg of a first-generation cephalosporin orally every 8 h for 7 days.^[14]

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Figure 3:

Post-operative and follow-up radiographs. (a) Immediate post-operative anteroposterior view of the pelvis showing the optimum position of the hip prosthesis bilaterally (on both sides, a ceramic femoral head of 32 mm was used in combination with polyethylene liners). (b) Plain radiographs at 6 weeks of follow-up. (c) Plain radiographs of follow-up at 11 months showing maintained implant positioning. (d) Last follow-up after 18 months showed preserved implants positioning.

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However, the post-operative hemoglobin (Hb) levels showed anemia, which was managed by blood transfusion and iron replacement therapy. She was discharged after 1 week, and her Hb level at discharge was 8 g/dL. Sutures were removed 2 weeks postoperatively, and the wound healed without any complications.

On the 3^rd post-operative week, the patient presented with painless hematuria; upon consulting the rheumatology team, they asked for some investigations which showed the following: D-dimer, 4921 ng/mL (elevated); antithrombin, 31%; aspartate aminotransferase, 152 U/L; urea, 121 mg/dL; creatinine, 0.83 mg/dL; C-reactive protein, 54.7 mg/L; Hb, 7.1 g/dL; white blood cell (WBC), 15 × 10⁹/L; and procalcitonin, 36.64 ng/mL (markedly elevated).

They suggested acute post-operative periprosthetic infection (PJI) or systemic inflammation as a flare of her autoimmune condition as the probable diagnosis, and the patient was admitted to the ICU. Before starting empirical antibiotic management, we performed bilateral image-guided hip aspiration to confirm the PJI diagnosis; however, the aspiration was dry.

After 1 week of management in the ICU (including broad-spectrum antibiotic administration), her laboratory markers improved, with a rapid decline in the procalcitonin levels, indicating a response to treatment.

At 11 months postoperatively, the patient walked unassisted and showed no signs of infection or other complications. As per the HHS, her functional outcomes were 87 and 83.7 for the right and left hips, respectively. The plain radiographs showed proper implant positioning [Figure 3b and c]. At the last follow-up of 18 months, the patient’s HHS improved to 91.7 and 92.7 for the right and left hips, respectively, and the implants’ positioning was maintained as shown in the plain radiographs [Figure 3d].

DISCUSSION

The present report offers a rare case of bilateral hip arthropathy secondary to a rare autoimmune disease (DALD), which was also associated with a picture suggesting inflammatory arthropathy combined with ONFH, probably secondary to steroid therapy. The hip condition was managed successfully by performing a simultaneous bilateral cementless THA and involving other medical specialties (rheumatology, internal medicine, and anesthesia) in the diagnosis and management journey.

ALPS is considered a rare genetic condition related to disorders in lymphocyte homeostasis. The condition is characterized by chronic (non-infectious and non-malignant) uncontrolled lymphocyte proliferation, which is usually presented with autoimmune manifestations, splenomegaly, and lymphadenopathy.^[15] Various management modalities were suggested for controlling symptoms related to ALPS, such as corticosteroids and immunosuppressants; however, in some cases, splenectomy might be required.^[16] Furthermore, DALD is a very rare variety of ALPS (having a worldwide prevalence of <1/1,000,000) characterized by decreased function of the Fas death receptor, predominantly hematological autoimmunity-related presentations such as hemolytic anemia, which sometimes might be lethal, splenomegaly, and lymphadenopathy, and its management is mainly with immunosuppressants.^[9]

The literature discussing the precautions and outcomes of performing total joint arthroplasty (Knees and hips) in patients with autoimmune and inflammatory arthropathy is well reported.^[17-19] However, to the best of our knowledge, reports on hip or knee joint arthroplasty in patients with ALPS or DALD are very scarce.

We believe that reporting such a case is beneficial to delineate the medical and surgical challenges surrounding such a rare autoimmune disease, which might include some of the following:

First, the diagnosis of such a rare condition necessitates collaboration with other medical specialties, and a dedicated diagnostic algorithm is beneficial [Figure 1]. Furthermore, for the musculoskeletal manifestations associated with ALPS or DALD, these might be related to their autoimmune nature, such as the development of inflammatory arthropathy or as a complication of the medical treatment, such as ONFH associated with prolonged steroid intake.^[20,21] This combination was noticed in the current case, where reduced hip joint space is a constitutional sign associated with inflammatory arthropathy, while the picture of ONFH as evident in the MRI is consistent with prolonged steroid usage.

Second, issues related to medical treatment being held before THA surgery, especially immunosuppressants, and whether the patient was on any biological treatments. Regarding this aspect, we relied on the recommendations reported by the ACR/AAHKS Guideline for tailoring the drugs perioperatively, where disease-modifying antirheumatic drugs could be safely continued and biologics should be stopped during surgery and continued at a minimum 14 days postoperatively; however, for steroids, the daily prednisone dose or its equivalent should be <20 mg.^[13]

Third, regarding the deep venous thrombosis (DVT) prophylaxis, we highlighted this issue in the current case, although we discussed the various options with the treating medical team; however, we did not change our usual protocol, which we practice for our usual patients. The literature reported a possibly higher incidence of DVT in patients with autoimmune diseases.^[22] However, in a study by Matta et al., who evaluated about one billion hospitalized patients for joint surgeries, they reported a similar post-operative risk of venous thromboembolic events (VTE) between patients who were diagnosed with rheumatoid arthritis and those who were not.^[23] Furthermore, Shi et al. reported that the risk of VTE was lower in patients who underwent THA for ankylosing spondylitis compared to patients who were diagnosed with OA.^[24]

Fourth, a crucial point when dealing with patients diagnosed with a kind of autoimmune disease is how to differentiate between the post-operative systemic inflammation flare and the acute onset of PJI. This issue has been discussed in various reports, with the conclusion that the diagnosis is difficult to achieve; however, considering the patient’s baseline inflammatory markers, synovial fluid WBC, and synovial fluid bacterial cultures are proper steps for PJI diagnosis.^[25-27]

Fifth, although performing bilateral THA in patients with bilateral hip diseases carries its own risk, it offers some benefits for the patients and the healthcare system.^[28] In a systematic review and meta-analysis by Ramezani et al., the authors reported that simultaneous bilateral THA had fewer complications than staged bilateral THA, further lowering the total cost.^[6] In a more recent study by Razzaghof et al., reporting the long-term results of performing simultaneous bilateral THA in patients with juvenile idiopathic arthritis, at a mean follow-up of 11.3 years, they reported HHS scores improvement from a mean 49.6 points preoperatively to a mean of 79.7 points postoperatively; furthermore, they reported no cases of dislocation, deep PJI, or revision, concluding the safety and efficiency of such procedure.^[7]

Sixth, selecting the proper surgical approach. In the current case, we acknowledge that performing the surgery through a direct anterior approach could have avoided the need to turn the patient to the other side to operate on the second hip through a lateral or posterior approach (as was done in the current case).^[7,8] However, due to a lack of experience and the severity of the hip deformity, we preferred proceeding with the surgical approach that is familiar to the surgical team.

Seventh, issues related to extensive soft tissue release. One of the technical difficulties we faced in the current case was exposure and mobilization of the hip, which was evident during the clinical examination as a profound limitation of hip motion. We performed a stepwise, extensive soft tissue release; the caveat of performing such a release is the possibility of post-operative THA instability; however, we did not encounter such a problem in the current case. Such a step is well described in the literature in cases with severe developmental hip dysplasia and cases with ankylosing spondylitis with no increased risk.^[29,30]

Last, regarding THA fixation options. In cases with relatively poor bone quality, a cemented fixation might be a suitable option; however, in the current case, considering the patient’s age and relatively good bone quality, we selected cementless fixation. Such a fixation was reported to have an acceptable long term results as reported by Simon et al., where the authors reported that in their series of 57 THAs (10 cemented and 47 cementless cups) in 32 patients who were under the age of 35 at the time of surgery, after an average follow-up of 12.2 years, they reported three aseptic loosening of the cemented cups and no revision was performed for the cementless cups.^[31] However, cemented fixation might be preferable if the surgeon faced poor bone quality and a wide femoral canal.^[17]

We admit that the current report is limited by the relatively short follow-up period, and a longer follow-up is required to report the longevity of the implants and the maintenance of the patient’s functional outcome improvement.

CONCLUSION

The current case report highlights the importance of working with other specialties to achieve proper patient diagnosis and management (pre-, intra-, and postoperatively). Autoimmune diseases, including the rarely occurring ones such as Dianzani lymphoproliferative disease, could present with a variety of hip symptoms secondary to the disease itself or as a complication of the medical treatments. Simultaneous bilateral THA will help the patient recover better.