18 Jun 2021

3/8/21: Cryoglobulinemic Vasculitis

PGY3 Michelle Banh presented a case of a 58 year old M with history of remote polysubstance abuse/IVDU, HFrEF (EF 47%), HTN and CKD Stage V who presents with b/l LE rash and edema x1 week.  About 3 weeks prior, patient was started on amlodipine 5mg qday for HTN.  After taking for 5 days, he noted b/l LE edema and appearance of new rash so he stopped amlodipine but rash didn’t resolve.  Rash is pruritic and associated with paresthesias.  He’s been applying OTC hydrocortisone cream to anterior shins daily without relief.  Denies preceding skin breakdown or trauma to Les.  Denies other new medications or herbal supplements.

ROS: + decreased UOP

Home Medications: Atorvastatin 20mg qhs, Carvedilol 50mg BID, Furosemide 80mg BID, Hydralazine 100mg TID, Isosorbide dinitrate 60mg TID

Vitals: T 36.9, HR 74, RR 16, BP 150/95,O2 sat 98% on RA

Physical Exam significant normal cardiac and pulm exam; 2+ edema up to bilateral mid-shins; skin with large flat, non-blanching purpura over bilateral lower shin sparing posterior compartment, some hyperpigmented areas within purpura (chronic per patient); large flat purpura over L medial thigh; scattered discrete, raised, blanching circular areas of erythema over abdomen (chronic per patient)

CBC: WBC 4.5> Hb 8.9/Hct 26.9 <Plt 156 (MCV 85, RDW 15.8%)

CMP:

Na 140/K 4.4/Cl 109/HCO3 18/BUN 57/Cr 4.5<Glucose 91 (Baseline Cr: 3.5-4)

ALP 58>T.protein 5.8/Albumin 3.7/AST 43/ALT 67/Tbili 0.5/Dbili 0.2

Ca 8.5

Coags: PT 14.7, INR 1.16, PTT 30.7

Renal workup significant for UA showing >300 proteins, large blood (>50 RBCs).  Further nephritic workup showed Urine protein/cr elevated at 3.71 and hepatitis panel positive for Hep C.  Renal vein duplex negative for thrombosis.

Infectious workup negative for HIV, RPR, Blood culture and viral studies (CMV, EBV and parvovirus).

Heme workup with APLS labs negative.

Rheum workup negative for autoimmune disease (negative ANA, ANCA, dsDNA).  Complement levels low.

Cryoglobulin screen positive with 4.1% detecting.

Bone marrow biopsy performed with no morphologic or immunophenotypic evidence to support lymphoblastic lymphoma or plasma cell myeloma.  Arteries showed fibrinoid necrosis with vaguely granulomatous features.  Given history of cryoglobulinemia, it is compatible with cryoglobulinemia vasculitis.

Treatment: Patient was discharged for further planning between Rheum and Hepatology for treatment as outpatient.

Teaching points:

  1. Consider vasculitis when patient presents with multiorgan involvement (renal, heme, derm, neuro).
  2. Use serologies to guide diagnosis, obtain tissue if possible.
  3. HCV can have intra- and extra-hepatic manifestations.
  4. Clinical triad of HCV related cryoglobulinemia: purpura, neuropathy, polyarthralgia.
  5. HCV treatment is mainstay of management of HCV related cryoglobulinemia.

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