Severe Disease Activity and Complications of Immunosuppressive Therapy: A Challenge for Acute Hospital-based Rehabilitation in Rheumatology

Acute Rehabilitation Cases from the Major Fields of Rheumatology

Case 1: Rheumatoid arthritis

With the resources of modern acute rheumatology it is possible to control high disease activity in RA. It is prolonged recovery from endoprosthetic replacement of the large joints or severe complications of immunosuppressive therapy or both that brings a patient in need of integrated acute and rehabilitative care. In longstanding RA, normal mobilization and rehabilitation are limited by the preexisting destruction and glucocorticoid-induced myopathy.

A 63-year-old woman with a 20-year history of RA (Figures 1A, 1B), endoprosthetic replacement of both knees in 1994–95 and the left hip in 2004, and spondylodesis of C0/C4 in 2001 and T12-L5 in 2000 (Figures 1C, 1D) was under treatment with methotrexate and leflunomide in combination with glucocorticoids (15 mg/day for more than 3 years). She was admitted directly to the ICU with respiratory insufficiency and renal failure due to sepsis caused by Staphylococcus aureus infection of the endoprostheses of both knees and the left hip. She underwent one-stage knee endoprosthesis exchange and creation of a Girdlestone situation in the left hip joint (Figures 1E–1G). After orthopedic intervention she was treated in the ICU for 12 days, followed by another 10 days on a sterile ward, before she was transferred for multidisciplinary rehabilitation to the ARU. The clinical examination revealed generalized muscle atrophy, reduced strength of the arms and legs (3/5 according to Janda of the arms and 2/5 of the legs). She suffered from glucocorticoid-induced myopathy and critical illness polyneuropathy. When she was transferred to our unit, she could not sit without support, stand, or walk. Transfer was not possible without assistance. She could not eat without assistance because she was unable to move her hands toward her mouth. The post-event Barthel index^10,11 was only 20 points. Before the septic event, she had been completely independent and did not need any assistance in daily living. Due to the marked loss of function, with reduced ADL, she experienced reactive depression. The aim of acute rehabilitation in this patient was to achieve stabilization to an extent that she could undergo surgical restoration of the left hip endoprosthesis. Her treatment consisted of daily fractionated individual therapy sessions, including physical exercises on a neurophysiological basis for stabilization of the trunk several times a day, transfer training assisted by 2 physiotherapists, respiratory therapy for pneumonia prophylaxis and to restore normal breathing, and occupational therapy for training in ADL. Altogether, she received 3–4 hours of individual training per day 7 days a week. The physicians caring for her included a specialist in physical and rehabilitation medicine, a rheumatologist, a pain therapist, and a psychiatrist. After 8 weeks of acute rehabilitation, she was able to undergo surgery and the planned hip endoprosthesis restoration was performed, including stabilization of the socket with a Burch-Schneider ring (Figure 1H). Postoperatively, a second phase of acute rehabilitation was indicated; the aim was to restore a partial loading capacity of 15 kg of the left leg. She received individual sessions with the same frequency as preoperatively to train in standing and walking, first with a hydraulic walking device, then with a walking frame. At the end of acute rehabilitation, she was able to transfer without assistance and had a walking distance of 20 meters with the hydraulic device and 10 meters with a walking frame. The Barthel index increased to 35 points at discharge. She was discharged to home rehabilitation after acute and post-acute rehabilitation was concluded.

• Download figure
• Open in new tab
• Download powerpoint

Figure 1.

A 63-year-old woman with rheumatoid arthritis. Radiographs of hands (A) and feet (B) show severe joint destruction and deformity. C. Status post C0–C4 stabilization. D. Status post T12-L5 spondylodesis. E, F. 3-D reconstructions of a computed tomography scan of the left hip showing Girdlestone situation and titanium cerclage of the fractured femur. G. Cranialization and demineralization of the Girdlestone femur. H. Radiograph after left hip endoprosthesis reconstruction with a Burch-Schneider ring (socket).

Case 2: Ankylosing spondylitis

A 29-year-old man (Figure 2A–2D) with a 7-year history of AS and endoprostheses of the hips lived in an adjustable easy chair for 2 years before admission to the hospital due to severe back pain. He left the chair only when his parents transferred him to the bathroom. He was treated with methotrexate, sulfasalazine, glucocorticoids, and nonsteroidal antirheumatic drugs. We selected this case because of the high disease activity, which led to complete immobility.

• Download figure
• Open in new tab
• Download powerpoint

Figure 2.

A 29-year-old man with ankylosing spondylitis. A. Lateral radiographic view of the cervical spine showing bamboo spine. B. 3-D reconstruction of a computed tomography scan of the lumbar spine and sacroiliac joints showing severe ankylosis. C. The patient on admission showing extreme obesity and psoriasis. D. The patient today.

On admission, he had fixed hyperkyphosis of the thoracic spine, extreme obesity (weight 115 kg, height 177 cm), and extensive psoriatic skin lesions of the trunk and in the genital area (Figure 2C). Radiography demonstrated the typical changes of AS in the cervical spine (Figure 2A). There was complete ankylosis of the sacroiliac joints on both sides (Figure 2B).

Treatment in the acute hospital setting was necessary because of the high level of impairment and also because strict monitoring of side effects of a new drug regimen was needed. Further, daily intensive skin care was performed in the department of dermatology.

He was admitted to the ARU because of functional loss and inability to perform ADL due to high disease activity and severe pain. In spite of intensive physical and occupational treatment, psychological therapy, and pain management, no substantial improvement was achieved due to the high activity of AS. The spinal form of psoriatric arthritis was discussed as a differential diagnosis. After initiation of treatment with the tumor necrosis factor-α inhibitor infliximab, an impressive reduction of disease activity and pain was observed. The full acute rehabilitation program with 6 hours of individual therapy could be started. The impressive gain in function achieved in this patient was only possible with the early onset of an integrated acute rehabilitation program. The aim of the program for this patient was to restore function to an extent that he could undergo further post-acute rheumatological rehabilitation on an outpatient basis (Figure 2D). This was achieved after 57 days of acute rehabilitation and a total of 97 days as an inpatient. He was able to do the transfers by himself and walked short distances with a hydraulic walking device. The Barthel index increased from 30 points on admission to 65 points at discharge from the ARU.

Case 3: Vasculitis

To illustrate the severe complications that may be associated with modern immunosuppressive therapy, we describe a 61-year-old woman with a 5-month history of high activity Wegener’s granulomatosis (Figures 3A–3C). Despite cyclophosphamide and glucocorticoid pulse therapy, followed by oral cyclophosphamide accompanied by high-dose glucocorticoids (up to 60 mg), the vasculitis activity remained high, with bronchial stenosis and mononeuritis multiplex resulting in paresis of the facial, glossopharyngeal, and recurrent nerves. Only B cell depletion with rituximab controlled the disease. One month after discharge from the hospital, the woman was hospitalized with severe respiratory insufficiency due to pneumonia (Aspergillus and Pseudomonas infection) and recurrent highly active vasculitis with new bronchial stenosis (Figure 3B). The 5-month course of high-dose glucocorticoids led to severe osteoporosis with fractures of the T8–T10 vertebrae (Figure 3D). Vertebroplasty was performed for pain management, but was complicated by embolism of a pulmonary artery (Figures 3E–3G), and the patient’s respiratory situation worsened. The complicated pulmonary situation with bronchial stenosis and mucus retention and reduced mobility due to severe back pain was the indication for transferring her to the ARU. Her treatment comprised intensive physical respiratory therapy including reflectory respiratory treatment several times per day to reduce mucus retention in order to prevent a new systemic infection with Pseudomonas, which was multiresistant by then.

• Download figure
• Open in new tab
• Download powerpoint

Figure 3.

A 61-year-old woman with a 5-month history of Wegener’s granulomatosis. A. Granulomas in the paranasal sinuses (arrowheads) and in the orbita (arrow). B. 3-D reconstruction of a computed tomography scan of the lungs showing stenosis of the right main bronchus (arrow). C. Fiberoptic bronchoscopic view showing ulcers, granulomatous changes, and fibrin covering. D. Magnetic resonance imaging scan showing steroid-induced vertebral fractures of T8–T10. E. Status post-vertebroplasty; displacement of bone cement into spinal canal and paravertebral veins shown by computed tomography (sagittal view). F. Magnified chest radiograph shows pulmonary artery embolism caused by bone cement (arrowheads). G. Lateral radiograph of the lumbar spine showing additional impression fractures.

The aim was to improve her condition to the point that she could be referred for further post-acute rehabilitation in a pulmonary facility, and this was achieved after 19 days of acute rehabilitation and a total hospital stay of 149 days. The Barthel index increased from 50 points on admission to 85 points at discharge from the ARU.

Case 4: Systemic lupus erythematosus

A 21-year-old woman had systemic lupus erythematosus with multiple gut perforations and severe acute pancreatitis. Acute treatment consisted of modified extended hemicolectomy on the left with blind closure of the descending colon, terminal stoma of the ascending colon, and lavage. The course was complicated by sepsis and multiple organ failure. Repeated laparotomies were needed because of abscess formation (Figure 4A).

• Download figure
• Open in new tab
• Download powerpoint

Figure 4.

A 21-year-old woman with systemic lupus erythematosus. A. Abscess (white arrow) adjacent to the descending colon (black arrow). Transverse (B) and sagittal (C) images of abdominal wall defect (arrowheads) after repeat laparotomy. D. Abdominal wall defect covered with an artificial mesh and colostomy with only a small tissue bridge between. E. Abdominal wall defect with bandage. F. Livedo reticularis, peroneal splints (critical illness polyneuropathy).

Mobile rehabilitation was started right after ICU treatment with 48 days of artificial respiration and a total of 14 laparotomies. A large defect of the abdominal wall covered only by an artificial mesh (Figures 4B–4E) and significant critical illness polyneuropathy complicated the course of acute rehabilitation.

When transferred to the ARU, she was able to walk a few steps with a hydraulic walking device. Unassisted transfer was not possible. Her Barthel index was 45 points. Acute rehabilitation comprised at least 4 treatment units per day with physical exercises and walking training with the hydraulic walking device, later with a walking frame and then forearm crutches. She received peroneal splints due to peroneal nerve paresis secondary to critical illness polyneuropathy (Figure 4F). The trunk was additionally stabilized with an abdominal bandage. Continual rheumatological monitoring was necessary. In addition, she received occupational therapy, pain therapy, and psychiatric and psychological treatment. A physician and a specialist nurse changed the bandage once a day. The patient started to learn stoma care. The primary aim of acute rehabilitation, early mobilization and mental stabilization, was achieved after 77 days. She had a Barthel index of 85 points when she was discharged from our ward. Because of de novo lupus activity, she was transferred back to the acute rheumatology ward to optimize antirheumatic drug therapy. One week later she was discharged home.

Time Requirements for Acute Rehabilitation

According to the guidelines of acute rehabilitation in Germany the necessity of acute medical care and the extent of functional loss due to disability or impairment are the admission and discharge criteria for acute rehabilitation. A widely used instrument for measuring functional impairment is the Barthel index.

In our multidisciplinary ARU, patients are usually treated until they have reached a functional level that they can undergo post-acute and longterm rehabilitation, i.e., they have a Barthel index > 70 points. Patients undergo acute rehabilitation typically after prolonged stays in the ICU with gastroenterological, cardiological, nephrological, and oncological underlying diseases, and also with sepsis and multiple organ failure, management after orthopedic operations, in particular endoprosthesis replacement surgery, and patients with functional deficits after stroke. The average time spent in the ARU was 37.9 days (standard deviation 10.1 days, range 9–106 days) between July 2006 and June 2007 (n = 143).

The hospital stays of 3 of the 4 rheumatological patients described here were markedly longer than the average (Table 1), because the patients had conditions with high disease activity or complications of immunosuppression, and the longer hospital stays reflect the complexity of acute rehabilitation. In these 4 cases, acute rehabilitation accounted for 13% to 67% of the time these patients spent in hospital. The functional features of all 4 patients on admission and at discharge from the ARU are summarized in Table 2.