mycophenolic-acid and Cryoglobulinemia

Autoimmune diseases are sometimes associated with immune-mediated renal diseases and cryoglobulinemia is one of the causes. Cryoglobulinemia and cryoglobulinemic glomerulonephritis associated with primary Sjögren's syndrome are most frequent condition among non-hepatitis C virus-related condition. Its typical renal manifestation shows high amount of proteinuria with microscopic hematuria and renal insufficiency. We describe a case of 72-year-old woman with Hashimoto disease, autoimmune hepatitis, Sjögren's syndrome, and immune-related pancytopenia complicated by cryoglobulinemic glomerulonephritis. Before kidney biopsy, tubulointerstitial nephritis probably due to Sjögren's syndrome was suspected because of persistent hematuria without significant proteinuria and developing mild renal dysfunction over 6 months. The developing renal dysfunction associated with isolated hematuria is uncommon in glomerular diseases. Kidney biopsy, however, revealed established membranoproliferative glomerulonephritis with subendothelial deposits consisting of tubular structures with IgM, IgG, and C3 staining. Corticosteroids plus mycophenolate mofetil therapy successfully normalized renal function. Physician should not overlook cryoglobulinemic glomerulonephritis, which is potentially poor prognosis, even if urinalysis shows only persistent isolated hematuria in patients with autoimmune diseases.

Topics: Adrenal Cortex Hormones; Aged; Antibiotics, Antineoplastic; Autoimmune Diseases; Cryoglobulinemia; Female; Glomerulonephritis; Glomerulonephritis, Membranoproliferative; Hashimoto Disease; Hematuria; Hepatitis, Autoimmune; Humans; Kidney; Methylprednisolone; Mycophenolic Acid; Nephritis, Interstitial; Pancytopenia; Renal Insufficiency; Sjogren's Syndrome; Treatment Outcome

To report a unique case of peripheral ulcerative keratitis secondary to hepatitis B virus (HBV)-associated cryoglobulinemia and vasculitis and its pharmacological and surgical treatment and 2-year follow-up.. A 52-year-old woman presented with unilateral eye pain and photophobia, arthralgia, remnants of a maculopapular rash, and subsequently facial numbness several weeks later. Her best spectacle-corrected visual acuity (BSCVA) in the affected eye was 20/80. Slit-lamp examination revealed severe superior corneal thinning without infiltrate. Corneal ulceration worsened until 10% of the cornea remained. Laboratory workup was positive for rheumatoid factor and revealed significantly decreased C4 complement, and HBV serology was positive.. Clinical history, examinations, and laboratory results suggest HBV-associated cryoglobulinemia and vasculitis. Management included prednisone, cyclophosphamide, and mycophenolate mofetil for immunosuppression and tenofovir for HBV treatment. Conjunctival resection and a glue patch were used to reduce inflammation and stabilize corneal melt. BSCVA improved after treatment was initiated. Two years after initial presentation, her BSCVA is 20/30, significantly improved from her vision at presentation.. Diagnosis of peripheral ulcerative keratitis requires thorough history and physical examinations given the numerous causes. Prompt treatment including immunosuppressive medication and, in this case, antiviral medication is crucial to preventing serious visual consequences including corneal perforation and blindness.

Topics: Antiviral Agents; Corneal Ulcer; Cryoglobulinemia; Cyclophosphamide; Female; Glucocorticoids; Hepatitis B, Chronic; Humans; Immunosuppressive Agents; Middle Aged; Mycophenolic Acid; Ophthalmologic Surgical Procedures; Prednisone; Tenofovir; Vasculitis

Cryoglobulinemic vasculitis is extremely rare in patients with systemic sclerosis (SSc). So far, only two cases of cryoglobulinemic vasculitis in SSc were described in the literature. This report is about a patient with SSc and secondary Sjőgren's syndrome, who developed typical clinical features of small-vessel vasculitis, including arthritis, purpura, microhaematuria, gangrene of fingers, and toes and myocardial ischemia, in the presence of mixed cryoglobulinemia, ANA, rheumatoid factor, and anti-SSA/Ro antibodies. Symptoms and signs of vasculitis worsened despite initial treatment with corticosteroids and cyclophosphamide, but improved significantly when mycophenolate mofetil was used instead cyclophosphamide.

Topics: Adrenal Cortex Hormones; Aged; Cryoglobulinemia; Cyclophosphamide; Drug Substitution; Humans; Immunosuppressive Agents; Male; Mycophenolic Acid; Scleroderma, Diffuse; Sjogren's Syndrome; Treatment Outcome; Vasculitis

Membranoproliferative glomerulonephritis associated with Type II cryoglobulinemia is the predominant type of HCV-related glomerulonephritis. Immunosuppressive and anti-viral therapy is alternately used to treat it, but the results are not always satisfactory or lasting. In this paper we report 3 cases of cryoglobulinemic membranoproliferative glomerulonephritis, treated with different and personalized therapeutic approaches by using conventional therapy and new drugs such as mycophenolate mofetil and rituximab. Our case series report emphasizes the importance of choosing the treatment for each patient, taking into account many factors: age, severity of liver and renal involvement, extra-renal manifestations, any previous treatment, contraindications or adverse events and last but not least the balance between immunosuppression and virus activity.

Topics: Adrenal Cortex Hormones; Aged; Angiotensin II Type 1 Receptor Blockers; Anti-Inflammatory Agents, Non-Steroidal; Antibodies, Monoclonal, Murine-Derived; Antiviral Agents; Biphenyl Compounds; Cryoglobulinemia; Female; Glomerulonephritis, Membranoproliferative; Humans; Immunologic Factors; Interferon alpha-2; Interferon-alpha; Irbesartan; Male; Middle Aged; Mycophenolic Acid; Polyethylene Glycols; Recombinant Proteins; Ribavirin; Rituximab; Tetrazoles

Topics: Cladribine; Cryoglobulinemia; Humans; Immunosuppressive Agents; Male; Middle Aged; Mycophenolic Acid; Treatment Outcome