Msk Dermatology · Year 2 · from Msk Dermatology

Case 2: Established Rheumatoid Arthritis with Extra-Articular Manifestations

Patient Presentation

Demographics: 58-year-old woman

Chief Complaint: "My RA has been flaring, and I've noticed increased shortness of breath."

History of Present Illness: The patient has a 15-year history of seropositive rheumatoid arthritis, initially well-controlled on methotrexate but with increasing disease activity over the past year. She has developed multiple subcutaneous nodules on her elbows and fingers. Over the past 4 months, she has noticed progressive exertional dyspnea, now occurring with walking one block. She has a dry, nonproductive cough. She also reports dry eyes requiring frequent artificial tear use and dry mouth making it difficult to eat crackers without water.

Past Medical History:

• Rheumatoid arthritis diagnosed at age 43
• Hypertension
• Osteoporosis (steroid-induced)
• Secondary Sjogren syndrome

Medications:

• Methotrexate 20 mg weekly (on for 15 years)
• Folic acid 1 mg daily
• Prednisone 5 mg daily (unable to taper below this)
• Lisinopril 10 mg daily
• Alendronate 70 mg weekly
• Artificial tears as needed

Social History:

• Never smoker
• Retired librarian

Physical Examination

• Vital Signs: BP 132/78 mmHg, HR 88 bpm, RR 18, SpO2 94% on room air, Temp 36.8C
• General: Appears chronically ill
• Lungs: Fine bibasilar crackles, no wheezes
• Cardiovascular: Regular rhythm, no murmurs
• Hands:
• Ulnar deviation at MCP joints bilaterally
• Swan neck deformity of bilateral 3rd and 4th fingers
• Reduced grip strength
• Multiple subcutaneous nodules on finger extensor surfaces
• Elbows: Large firm nodules over bilateral olecranons
• Eyes: Mild conjunctival injection
• Mouth: Reduced saliva pooling, mild tongue fissuring

Workup and Results

Laboratory Studies:

Pulmonary Function Tests:

• FVC: 68% predicted (reduced)
• FEV1: 72% predicted
• FEV1/FVC: 0.82 (normal ratio)
• DLCO: 52% predicted (significantly reduced)
• Pattern consistent with restrictive lung disease

Imaging:

• High-resolution CT chest:
• Bilateral reticular opacities, predominantly basilar
• Ground-glass opacities
• Traction bronchiectasis
• Pattern consistent with usual interstitial pneumonia (UIP) or nonspecific interstitial pneumonia (NSIP)
• Hand radiographs:
• Bilateral erosions at MCP and PIP joints
• Joint space narrowing
• Subluxation at multiple MCP joints

Clinical Image

High magnification histology of a rheumatoid nodule showing central fibrinoid necrosis surrounded by palisading histiocytes (macrophages), characteristic of rheumatoid nodules. Source: Wikimedia Commons, CC BY-SA 3.0.

Diagnosis

Established Rheumatoid Arthritis with:

1. Rheumatoid interstitial lung disease (ILD) - UIP or NSIP pattern
2. Rheumatoid nodules (multiple)
3. Secondary Sjogren syndrome (sicca symptoms with anti-Ro/La antibodies)
4. Anemia of chronic disease
5. Joint deformities (ulnar deviation, swan neck)

Treatment Plan

Address Interstitial Lung Disease:

1. Pulmonology referral for further evaluation and co-management
2. Consider mycophenolate mofetil: May benefit both joints and ILD; methotrexate-associated pneumonitis must be excluded
3. Discontinue methotrexate: Concern for possible contribution to lung disease (though methotrexate pneumonitis is typically acute)
4. Consider nintedanib: Antifibrotic approved for progressive fibrosing ILD

Modify RA Therapy:

1. Add rituximab: B-cell depletion may benefit both RA and ILD, particularly with positive anti-Ro antibodies
2. Continue low-dose prednisone: May benefit both conditions
3. Avoid JAK inhibitors: Concern for VTE risk, and less data for ILD

Manage Sicca Symptoms:

1. Continue artificial tears, consider punctal plugs
2. Saliva substitutes, sipping water
3. Consider pilocarpine or cevimeline if inadequate

Other Measures:

1. Pneumococcal and influenza vaccination (if not up to date)
2. Continue osteoporosis treatment
3. Screen for depression (chronic disease burden)

Teaching Points

1. Pulmonary disease in RA: ILD is a major cause of morbidity and mortality; screen with PFTs and HRCT when symptomatic; UIP pattern carries worse prognosis than NSIP
2. Rheumatoid nodules: Occur almost exclusively in RF-positive patients; paradoxically, methotrexate can cause accelerated nodulosis in some patients
3. Secondary Sjogren syndrome: Common overlap; sicca symptoms plus anti-Ro/La antibodies; increases lymphoma risk
4. Treatment complexity: ILD may require switching away from methotrexate; mycophenolate and rituximab have potential benefit for both joints and lungs
5. Anemia of chronic disease: Common in active RA; normocytic with low iron but elevated ferritin; responds to inflammation control rather than iron supplementation

All cases for this lecture as Markdown