One Year Therapy with Cyclosporine A is Useful in Prevention of Early Autoimmune Transformations in Predisposed Patients for Type I Diabetes Mellitus

Type 1 diabetes (T1D) is a chronic autoimmune disordercharacterized by specific immune destruction of the insulinproducing pancreatic β-cells. The loss of β cells involves bothgenetic and ill-defined environmental factors. The highest riskHLA-DR3/4 DQ8 genotype has been shown to be highlyassociated with β-cell autoimmunity and benign insulinitis.Subsequently; catalysts viz. viruses transform predisposedsubjects into overt diabetics. Such autoimmunity is herald byautoantibodies and subsequently low C-peptide production.Hence, there is no ideal autoimmune therapy for T1D sincepatients are genetically predisposed. Previous autoimmunemeasures to suppress early transformations were crippled bylong-term complications of medications. In our case report; welimited the use of Cyclosporine A to 1 year in a patient and itwas cost effective.

Idiopathic Interstitial Nephritis: An Autoimmune Disease with a Chameleon Presentation and High-Potential for Kidney Loss

Data on the clinical picture, prognosis and management of 12cases of idiopathic interstitial nephritis (IIN) are presented.Clinically; 5 patients had acute renal failure (ARF) with suddenfluid overload and azotemia, 2 had ARF on top of chronicrenal disease (CRD) and 6 with unexplained CRD. Theoutcome of the 5 patients with ARF and short duration ofillness was better. One had infrequent relapses, 1 hadfrequent relapses and 3 were steroid-dependent. The latter 4patients had required maintenance immunosuppression (IS)initially with Prednisone, Mycophenolate and finally withRituximab. The 2 patients with ARF on top of CRD werestabilized after IS. The remaining 5 patients with CRD becamestable and/or improved while 1 patient progressed to endstage kidney disease since her IS was late and inadequateprior to inclusion with us. In conclusion: IIN can mimic anykidney disease and can only be diagnosed with kidney biopsy.Aggressive IS can control the disease at its early stagesotherwise, the prognosis is poor.

Rituximab Treatment for Recurrent Skin Vasculitis Due to Cryoglobulinemia Associated with Hepatitis C Despite Viral Clearance with Sofosbuvir

Hepatitis C (HC) is a major cause of both acute and chronichepatitis. It is a major cause of liver cell failure and its cancer.Nearly 2/3 of patients experience extrahepatic immune-relatedmanifestations including skin vasculitis with/without mixedcryoglobulinemia. Naturally; clearance of viral infection shouldlead to prevention of such complications. Unfortunately; this isnot absolute true. In this case report; we describe a patient,with chronic HC infection yet without chronic liver disease, whocontinued to develop skin vasculitis and cryoglobulinemiadespite virological clearance of RNA virus by Sofosbuvir. Herskin vasculitis improved with yearly Rituximab infusions withoutactivation of hepatitis C infection, other autoimmune diseaseand hepatitis.

Acute Renal Failure Due to Cast Nephropathy without Multiple Myeloma

We report a 64 year-man with acute renal failure on top of hischronic renal disease due to diffuse diabeticglomerulosclerosis. Serum creatinine had reached 600umol/L from a baseline of 160 umol/L 4 months ago. Kidneybiopsy revealed diffuse broad casts obstructing the distaltubules with interstitial fibrosis associated with cast’sextravasation. The casts stained positive with kappa and notlambda indicating their monoclonal origin. The patient did nothave evidence of lymphoprolifertive disease and myeloma inparticular with normal skeletal survey and bone marrowtrephine biopsy. Moreover, serum protein and urineelectrophoresis failed to show monoclonal band. Only, directserum testing for free light chain confirmed high Kappa andnormal Lambda levels. The patient already had improved oncorticosteroids and subsequently Melphalan was added.Serum creatinine decreased to 180 umol/L by the end of this6-month treatment. The case illustrates a unique presentationof cast nephropathy without overt lymphoprolifertive disorderand with normal serum as well as urine proteinelectrophoresis. It emphasizes the need for kidney biopsyand/or direct measurement of serum free light chains fordefinitive diagnosis in such covert cases.

Hypercalcemic Crisis and Severe Renal Failure Due to Sarcoidosis Followed by Autoimmune Thrombocytopenia on Remission: A Case of Drug-Refractory and Chronic Disease

A 70-year-old woman, with a history of type II diabetesmellitus, presented with a 1-month history of progressiveweakness, disorientation and decrease appetite. She wasfound to have high serum calcium at 3.4 mmol/L and severerenal failure with serum creatinine at 700 umol/L. She hadnormal kidney ultrasound, vitamin D2, parathyroid hormoneand negative Quantiferon and Brucella tests. PET scanshowed increase uptake in the paratracheal lymph nodes.Mediastinoscopic lymph node biopsy disclosed non-caseatinggranulomata. Serum calcium and subsequently her acuterenal failure had improved with corticosteroids. She could nottolerate Azathioprine, Cyclophosphamide, andMycophenolate for severe gastrointestinal side effects.Moreover, her disease did not respond to Cyclosporine A,Methotrexate, Hydroxychloroquine and Rituximab.Ultimately, she was kept on Prednisone 20 mg daily as along-term maintenance therapy. Eight months afterremission, she developed severe thrombocytopenic purpura.Fortunately, the latter was controlled with temporary increasein her corticosteroid dose for 6 weeks. After 2 years oftreatment, she had spontaneous remission and Prednisonewas discontinued. No relapse was reported 1 year later. Inconclusion: Sarcoidosis can induce hypercalcemic crisis,acute renal failure and thrombocytopenia with activity up to 2years and treatment limited only to Corticosteroids.

Adalimumb Induced Systemic Vasculitis Following Treatment of Hidradenitis Suppurativa

A 52-year-old man, with diabetes mellitus and obesity, hadreceived Adalimumb for extensive Hidradenitis suppurativa.The latter was difficult to control with local antiseptics,antibiotics and surgery. Two weeks later, he developed fever,palpable maculopapular rash and progressive renal failure. Hisskin biopsy showed leucocytoclastic vasculitis with negativeimmune deposits and his kidney biopsy showed crescenticglomerulonephritis. Adalimumb was discontinued and thepatient was treated with Prednisone 1 mg/kg/day. His kidneyfailure improved. However, his skin lesions progressed tonecrotizing fasciitis despite aggressive surgical care.Ultimately, he died from disseminated sepses 2 months later

Rituximab Therapy for Idiopathic Chronic Urticaria with Recurrent Angioedema

We report on 4 patients with severe and idiopathic chronicurticaria with recurrent attacks of angioedema. Initially, theyresponded initially to high dose corticosteroids yet relapsed onhigh-maintenance dose. Subsequently, trial to use steroidsparing drugs viz. intravenous immunoglobulin thenmycophenolate mofetil with hydroxychloroquine and tacrolimushad failed to maintain a remission. Hence, Rituximab wasgiven. One month after Rituximab infusions, Prednisone dosewas successfully tapered down and was discontinued.Subsequently, all patients remained in complete remission for14-24 months of follow up.