Rheumatology‎ > ‎

Vasculitides / Including PMR

    • Non-palpable
      • Primary Cutaneous Ds (Trauma, Solar Purpura, Steroids)
      • Systemic Ds 
        • Coagulation abnormalities (Thrombocytopenia, Abnormal platelet function, Warfarin reaction, clotting factor defects, DIC)
        • Vascular Fragility (Scurvy, Amyloidosis, Ehlers-Danlos syndrome)
        • Emboli (Fat, cholesterol) 
    • Palpable(due to vessel wall inflammation)
      • Infection (Endocarditis, RMSF, Disseminated Gonoccocemia, Acute Menigococcemia)
      • Non-infectious inflammatory i.e Vasculitis (see below in vasculitis)
    • References: 

      Case 22-2011: A 79-Year-Old Man with a Rash, Arthritis, and Ocular Erythema (Small Vessel Vasculitis)

      Pathophysiology of Vasculitides (Harrisons')
      3 main mechanisms 
      • Pauciimmune or ANCA associated : GPA, E-GPA, MPA
      • Immune Complex formation or deposition: HSP(IgA), SLE, Hep-B, Hep-C Cryoglubunemic, Serum sickness with cutaneous vasculitis)
      • Pathogenic T- Lymbhocytes and granuloma formation: Giant cell, Takayasu, GPA, E-GPA


      Case 7-2015: A 25-Year-Old Man with Oral Ulcers, Rash, and Odynophagia

      • Type
        • Type I (M spike +, RF -)
        • Type II (M spike +, RF +)
        • Type III (M spike -, RF +)
      Lancet 2012
      • 2 main pathogenesis leading to organ damage and clinical manifestations
        • Cryoglobulinaemic Vasculitis: Immune-complex mediated activation of classic complement pathway (usually by type II i.e Mixed cryo)
        • Hyper-viscosity syndrome, leading to Vascular slugging, mainly by type I Cryo
          • Neurological (headache, confusion)
          • Ocular (blurry vision, visual loss)
          • Rhino-otological (epistaxis, hearing loss) 
          • Rapidly progressive Renal Failure 
          • Treatment:
            • Urgent 
            • Needs Plasma Exchange 
        • IgM and IgG can only activate complement system, hence cryoglobulinemic disorders are either IgG or IgM mediated.

      The most common clinical manifestations of cryoglobulinemic vasculitis are cutaneous vasculitis, arthritis, peripheral neuropathy, and glomerulonephritis. Renal disease develops in 10–30% of patients. Life-threatening rapidly progressive glomerulonephritis or vasculitis of the CNS, gastrointestinal tract, or heart occurs infrequently.
      • Clinical feature:
      • When to suspect 
      Lancet 2012

      Appreciate that all 3 Cryoglobulinemia types (Type I, Type II, Type III) as a cause Immune-Complex-mediated GN with low complement levels as a cause of RPGN

      This is an example of cryoglobuminemia as an example of complement mediated disease causing RPGN.
      Appreciate in B:
      • There is IgM, Kappa and IgG bands that are prominent. IgM, and Kappa has 2 prominent bands each. Likely 2 clones are involved. IgG on the other hand is polyclonal. 
      • Because this patient has M-spike (IgM, Kappa), and IgG is also present it is type II (Mixed) Cryoglobunemia.