An unusual duo: Immunodeficiency disorder and scleroderma

Area of Science:

• Immunology
• Rheumatology
• Infectious Diseases

Background:

• Human immunodeficiency virus (HIV) infection can present with various musculoskeletal manifestations.
• Managing autoimmune diseases in immunocompromised patients, such as those with HIV, poses significant clinical challenges.

Observation:

• A 45-year-old woman with a 10-year history of HIV on antiretroviral therapy presented with Raynaud's phenomenon and skin hyperpigmentation.
• She was diagnosed with pulmonary arterial hypertension and exhibited sclerodactyly and salt-and-pepper pigmentation.
• Investigations revealed a positive antinuclear antibody and a skin biopsy consistent with systemic sclerosis.

Findings:

• The patient was diagnosed with systemic sclerosis, an autoimmune connective tissue disease, co-occurring with HIV infection.
• A homology between HIV-1 and centromere B protein (CENP B) has been identified, suggesting potential pathogenetic links.

Implications:

• This case underscores the complex interplay between immunodeficiency and autoimmunity.
• It emphasizes the diagnostic and therapeutic difficulties in differentiating and managing conditions like pulmonary hypertension in HIV-positive individuals with coexisting autoimmune diseases.