乌鸦传媒

"Medical Journeys" is a set of clinical resources reviewed by physicians, meant for the medical team as well as the patients they serve. Each episode of this journey through a disease state contains both a physician guide and a downloadable/printable patient resource. "Medical Journeys" chart a path each step of the way for physicians and patients and provide continual resources and support, as the caregiver team navigates the course of a disease.

This month: A noteworthy case study.

What to do for this 70-year-old man with a 13-year history of poorly controlled rheumatoid arthritis (RA) when he develops severe arthritis pain in his shoulder?

That's what clinicians in Brazil needed to determine, as Camille Pinto Figueiredo, MD, PhD, of Universidade Católica de São Paulo in Sorocaba, Brazil, and colleagues reported in .

As they related in their case report, when the patient was diagnosed in 2010 tests had revealed elevated titers of rheumatoid factor and antibodies against cyclic citrullinated peptides. In the time since, his disease proved to be resistant to a range of previous treatments, including "corticosteroids and approved doses of hydroxychloroquine for 2 years, methotrexate for 3 years, leflunomide for 7 years, and adalimumab for 6 years."

Treatment with corticosteroids resulted in osteonecrosis of the hip, for which he underwent total arthroplasty.

In 2018, the team noted, the patient was admitted to hospital when he developed acute interstitial lung disease (ILD), which was diagnosed as organizing pneumonia. To address the possibility that his disease-modifying antirheumatic drugs and biologic therapies might be linked with the ILD and concomitant infection, these therapies were discontinued for 2 months.

At that point, the patient received only high glucocorticoid doses (methylprednisolone 80 mg/day with progressive dose reduction) to manage his RA and the lung disease.

A few months later, he presented with "a persistent flare with symmetric polyarthritis in peripheral joints and a severe painful bilateral shoulder arthritis," Pinto Figueiredo and co-authors said.

An assessment indicated a high level of disease activity, with a disease activity score in 28 joints (DAS28) of 6.42. An showed significant bone destruction: "Anteroposterior radiograph expansive intramedullary osteolytic lesion, at the metaepiphysis region of the humerus, with well-defined contours and sclerotic border, presenting internal septations. The image also shows an erosive lesion on the superolateral region of the humeral head, with slightly cortical thinning alongside," the team wrote.

A confirmed the findings and provided a more detailed description of the lesion, which included erosions related to a giant humeral head geode, along with synovitis, a large bursitis, and tendinopathy.

Clinicians prescribed a regimen of sulfasalazine up to 3 gm/day and intravenous abatacept (750 mg/dose), which was continued for 10 months. Nevertheless, the severe pain in the patient's shoulder and hands persisted, with a DAS28 of 5.45.

This was "associated with high levels of inflammatory markers (C-reactive protein [CRP] up to 3.76 mg/dL and erythrocyte sedimentation rate [ESR] up to 106 mm/1 hour), and functional impairment," Pinto Figueiredo and co-authors noted.

They switched the patient from abatacept to intravenous tocilizumab 8 mg/kg and administered "intra and periarticular corticosteroid injection in the left shoulder and intra-articular injection in both wrists." Treatment with leflunomide 20 mg/day was also resumed. In the following months, the patient achieved pain control, normal levels of inflammatory markers (CRP<0.03 mg/dL and ESR=3), and disease remission (DAS28=1.75).

Discussion

Rheumatoid arthritis involving the shoulder is seen much less commonly than involvement of the hands, feet, or wrists, which makes this case of "radiological progression with erosive damage and a giant geode, in an aggressive uncontrolled disease, even more unique," the authors said. MRI findings that revealed both bone and soft tissue damage confirmed the diagnosis.

One found that with or without involvement of other joints, shoulder arthritis affected 12.6% of RA patients, with associated symptoms more commonly observed in patients over age 60.

In a 2003 of shoulder involvement in 43 patients with RA, x-rays revealed that erosions affected the humeral head in 26 patients and the glenoid fossa in 12 patients. "On the other hand, MRI was able to detect erosions in glenohumeral joint in 39 patients and cystic lesions in 15," Pinto Figueiredo and co-authors said.

They explained that differential diagnoses for cystic lesions of the glenohumeral joint vary and include a range of conditions, including hallmarks of osteoarthritis such as joint space narrowing, subchondral bone sclerosis, marginal osteophytes, and subchondral radiolucencies. Solitary or large cysts only occur rarely.

"Gout manifests radiographic alterations as a late-stage effect of the disease, with deposits of urate crystals or tophi causing erosions of the underlying bone that resemble geodes or cysts," the case authors wrote.

They noted that although intraosseous ganglia tend to manifest on their own, as "unilocular or multilocular cystic lesions in the epiphysis of long bones," joint degeneration may lead to formation of cystic bone lesions in patients with septic arthritis, synovial chondromatosis, pigmented villonodular synovitis, and amyloidosis.

"Thus, MRI was of considerable assistance in the reported case," the team said.

The recent development of several biologic drugs used in to treat RA have made a significant impact due to their ability to control disease activity and decrease joint damage, Pinto Figueiredo and co-authors explained. These biological DMARDs (bDMARDs) have demonstrated the capacity to "inhibit damage installation and progression in peripheral small joints, as well as in large joints of lower extremities such as hip and knees."

This patient achieved disease control after "the association of conventional and an anti-interleukin-6 receptor biological DMARD, the team pointed out. "There are no data of -specific effects in shoulder joints of RA patients."

Conclusion

In this "rare clinical presentation of a giant geode in the humeral head ... MRI proved to be a valuable imaging method for identifying damage in both bone and soft tissue, supporting clinical data when other hypotheses may be implicated," the case authors concluded.

Read previous installments of this series:

Part 1: RA Beginnings: Before the Painful Joints

Part 2: RA: Still a Clinical Diagnosis

Part 3: RA: Choosing Initial Treatment