Case Series: Long-standing Untreated/Inadequately Treated Seropositive Rheumatoid Arthritis in the Golden Age of Disease-modifying Antirheumatic Drugs

Summary: Cristine K. Arcilla, MD, Gurjit Kaeley, MD, Myinth Thway, MD Rheumatoid Arthritis ( RA) is a systemic autoimmune disease that causes joint damage in 80% of the untreated patients but also other systemic complications. With the advent of multiple disease-modifying antirheumatic drugs (DMARDs), the clinical course, disease activity, and comorbidities from RA have significantly improved in the past few decades. Here we report a case series of three patients presenting to our institution with devastating complications of long-standing untreated/inadequately treated seropositive RA. Case 1 60-year-old female with a medical history of erosive RA, and interstitial lung disease (ILD) for more than ten years presented for progressively worsened shortness of breath and significant hypoxia. The patient was previously on biologic DMARDs, which she stopped believing worsened her symptoms. She denied urinary or fecal incontinence. Examination was remarkable for acute respiratory distress on high-flow oxygenation, diffuse rhonchi, and severe deformities and synovitis of major joints with impaired range of motion. Echocardiography revealed septal flattening concerning pulmonary hypertension. Chest imaging showed usual interstitial pneumonia (UIP) with new ground glass opacities in all lobes. Previous cervical imaging 3 months ago revealed extensive severe C1-C2 erosions with retrodental pannus with effusion. She is at high risk of myelopathy, quadriplegia, and death with procedures requiring cervical manipulation in the course of worsening respiratory failure. Fig. 1 MRI of the cervical spine revealed extensive severe C1- C2 erosions with retrodental pannus with effusion and multilevel stenosis. Case 2 69-year-old female with a medical history of RA for more than 30 years and perforated corneal ulcer of the left eye secondary to peripheral ulcerative keratitis presented for progressive right eye pain, redness, and photosensitivity with acute loss of vision. She was previously on DMARDs and steroids for a few weeks, which she stopped due to fear of side effects. Examination was remarkable for bilateral conjunctival injection and corneal clouding with discharge, severe joint deformities, and several severe synovitis. Slit lamp examination showed greater than 80% thinning of a peripheral ulcer/melt without infiltrate with fluorescein staining over a small area of corneal thinning on the right eye and diffuse stromal scarring inferiorly on the left eye. High sensitivity c-reactive protein (33 mg/L) and sedimentation rate (81 mm/hr) were elevated. Methylprednisolone 1g/day for three days was initiated and discharged with Prednisone 1 mg/kg taper. Follow-up ophthalmology exam showed a perforated ulcer of the right eye requiring glue and bandage contact lens. Infliximab infusion was contemplated, and she is agreeable. Fig. 2 Physical examination and slit lamp examination showed conjunctival injection with greater than 80% thinning of a peripheral ulcer/melt without infiltrate with fluorescein staining over a small area of corneal thinning on the right eye. Photo credits from Moody, Braeden, M.D. Case 3 73-year-old female with medical history of RA for more than 20 years, ILD-UIP, and atrial fibrillation on anticoagulation presented for severe right shoulder pain and swelling. She was lost to follow-up for four years and took DMARDs inconsistently. Examination was notable for severe hand and wrist deformities and bilateral shoulder fluctuance, right greater than left. Right shoulder radiography revealed increasing collapse and erosive changes of the humeral head and glenoid with prominent soft tissue swelling in the lateral. Right shoulder ultrasound showed marked destruction of the humerus along with multiple tendon tears and large effusion. Initial aspiration of the joint revealed 2 ml of straw-colored cloudy fluid. Investigated synovial fluid analysis for infection before initiation of Rituximab. Fig 3. Right shoulder radiography showed increasing collapse and erosive changes of the humeral head and glenoid with prominent soft tissue swelling in the lateral. We present a case series of three patients with rare, severe clinical presentations of RA. This emphasizes the huge disease burden and impact of RA on patients’ quality of life, including high risk of morbidity and mortality, permanent vision loss, and other severe organ manifestations as part of the natural course and progression of RA without DMARDs treatment. Patient counseling regarding the benefits over risks of treatment is central to RA management.