Giant cell arteritis in patients with systemic sclerosis: a case series

Area of Science:

• Rheumatology
• Internal Medicine
• Vasculitis

Background:

• Giant cell arteritis (GCA) and systemic sclerosis (SSc) are distinct autoimmune conditions.
• The co-occurrence of GCA and SSc is exceptionally rare, with limited understanding of optimal management.
• Defining treatment strategies for GCA in SSc patients is crucial due to potential overlapping symptoms and complications.

Purpose of the Study:

• To describe the largest single-institution case series of patients diagnosed with both GCA and SSc.
• To analyze the clinical presentation, diagnostic methods, treatment approaches, and outcomes in this rare patient cohort.
• To investigate the safety of glucocorticoid treatment for GCA in patients with SSc, particularly concerning scleroderma renal crisis (SRC).

Main Methods:

• Retrospective review of patients with diagnosis codes for both SSc and GCA from 1996 to 2020.
• Inclusion criteria required a formal diagnosis of both SSc and GCA by a rheumatologist.
• Data abstracted included demographics, clinical features, diagnostic modalities, treatments, and patient outcomes.

Main Results:

• Eight female patients were identified, with most meeting classification criteria for both GCA and SSc.
• Three patients with a history of SRC did not experience new SRC episodes after initiating glucocorticoids for GCA.
• Tocilizumab was used in three patients; one experienced a serious complication, one discontinued due to unrelated issues, and one achieved remission.

Conclusions:

• This case series represents the largest reported cohort of patients with concomitant GCA and SSc.
• Glucocorticoid therapy for GCA appears safe regarding SRC precipitation in SSc patients, even those with a prior SRC history.
• Further research is warranted to evaluate the efficacy and safety of tocilizumab in managing GCA within the context of SSc.