SHM Converge Scientific Abstract ePoster Gallery-Thicker Than Water: A Rare Case of Hyperviscosity Syndrome

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This case describes a 19-year-old female with a year-long history of weight loss, rash, epistaxis, and gingival bleeding who was found to have hyperviscosity syndrome. On exam, she had a low BMI, a hyperpigmented reticular/lacy rash, and mild splenomegaly. Labs showed microcytic anemia, marked rouleaux formation, elevated total protein with very low albumin, high inflammatory markers, and extremely elevated immunoglobulins (especially IgM) without a monoclonal spike on SPEP. Her serum viscosity was significantly elevated at 6.34.<br /><br />Given the bleeding symptoms and retinal findings of dot-blot hemorrhages, therapeutic plasmapheresis was started. Her elevated creatine kinase and aldolase, along with positive autoimmune serologies, led to a muscle biopsy, which showed necrotizing inflammatory myopathy with severe lymphocytic infiltrates in perivascular/perimysial spaces. She was treated with steroids and improved clinically and biochemically.<br /><br />The case is unusual because the patient had multiple positive autoimmune markers, including RF, anti-CCP, ANA, RNP, and other antibodies, but lacked enough classic clinical features to clearly fit a single rheumatologic diagnosis. Bone marrow and lymph node studies were unremarkable, and infectious workup was negative. The current working diagnosis is idiopathic inflammatory myopathy or mixed connective tissue disease (MCTD).<br /><br />The report emphasizes that polyclonal hyperviscosity can occur in autoimmune diseases such as rheumatoid arthritis, Sjögren syndrome, and IgG4-related disease, but myositis-associated hyperviscosity is rare and can create a challenging diagnostic and therapeutic problem.

Asset Subtitle

Ian Mines

Meta Tag

Author List Alaq MA Al-Abayechi, Esteban Toro Velez, Ian Mines, Sara Alhaj, Zohra Nooruddin

Category Clinical Vignettes

Concept Hyperviscosity Syndrome

Concept Polyclonal Immunoglobulin Elevation

Concept Monoclonal Gammopathy

Concept Rheumatologic Disease

Concept Myositis

Distinguished Non-Finalist

Presenter Organization University of Texas Health Science at San Antonio

Presenting Author Ian Mines

Track Adult

Keywords

hyperviscosity syndrome

polyclonal hypergammaglobulinemia

autoimmune myopathy

mixed connective tissue disease

necrotizing inflammatory myopathy

plasmapheresis

rouleaux formation

elevated IgM

retinal hemorrhages

rheumatologic antibodies

Hyperviscosity Syndrome

Polyclonal Immunoglobulin Elevation

Monoclonal Gammopathy

Rheumatologic Disease

Myositis