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Sclerodactyly for further evaluation; rheumatoid factor and Anti-CCP negative; chest x-ray no ILD; treatment started under my thickening of the skin
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Rheumatic & Autoimmune Conditions
Question #10794
1 year ago
669

Sclerodactyly for further evaluation; rheumatoid factor and Anti-CCP negative; chest x-ray no ILD; treatment started under my thickening of the skin

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Sclerodactyly for further evaluation; rheumatoid factor and Anti-CCP negative; chest x-ray no ILD; treatment started under my care January 2023; ANA profile negative; the phenotype of the disease is like scleroderma with sclerodactyly and thickening of the skin in the chest and the neck; not able to make a fist; start Mycophenolate in March 2023; September 2023 reports no improvement in skin stiffness and tightening and joint pain; consider Rituximab after reports-- RP 11 and RP 155 both 3 + strong positive; ; advised Rituximab INJ. Toritz RA 1 gm first dose in JAN 2024; second dose MARCH 2024;

Age: 45
Chronic illnesses: Sclerodactyly for further evaluation; rheumatoid factor and Anti-CCP negative; chest x-ray no ILD;
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Doctors' responses

This appears to be a scleroderma spectrum disorder with sclerodactyly and RP11/RP155 positivity, refractory to mycophenolate, now on Rituximab. Monitoring should focus on skin score, muscle enzymes, and PFTs. If Rituximab shows insufficient response after a few months, consider biologic escalation or overlap-targeted treatment.

Supportive Measures: Hand therapy and physiotherapy crucial for maintaining mobility Vitamin D3 and calcium if on steroids Low-salt, anti-inflammatory diet

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Given the detailed information you’ve provided about your condition, it sounds like you’re dealing with a complex case of the Scleroderma spectrum, even though key autoantibodies like ANA, rheumatoid factor, and Anti-CCP have come up negative, which can happen. The strong positive results for RP 11 and RP 155, though not commonly used in diagnosing scleroderma directly, suggest atypical autoantibody activity that still fits within a broader autoimmune disorder framework. Since Mycophenolate hasn’t provided the expected relief from skin stiffness and joint pain over several months, the shift to Rituximab seems a reasonable next step. Rituximab targets B cells that may be contributing to your symptoms and is often used in scleroderma cases where inflammatory and fibrotic processes are involved, though responses can vary from patient to patient. The plan to administer two doses in 2024 suggests an effort to target B-cell depletion more aggressively. It’s crucial to closely monitor for any side effects of Rituximab, such as infusion reactions or increased risk of infections, given its immunosuppressive nature. Additionally, you should maintain regular follow-up appointments with your care team, and potentially consult with a rheumatologist and a dermatologist to further refine and optimize your treatment strategy. Managing skin care with moisturizers to prevent cracking, staying physically active within comfort levels to maintain mobility, and watching out for new or worsening symptoms will aid in handling your daily routine more comfortably. If symptoms such as significant shortness of breath, unexplained weight loss, or severe pain appear or escalate, report them to your healthcare provider promptly to address any potential complications swiftly.

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