short case presentation

1. short case presentation HamdyKorayem ; MD Prof of Rheumatology - Alexandria

2. 55 year old male teacher , married and has 2 children. Presented complaining of: • Headache & easy fatigue of 5 months duration • Hand swelling & small hand joints pain.

3. On examination: • Patient looks conscious, alert, cooperative but he looks pale. • Vital signs :Blood pressure 200/100, heart rate 88 beats/min. • Chest & heart: clinically free. • Abdomen: clinically free with no organomegally. • Musculoskeletal examination: normal.

4. Past history: • Not diabetic. • No allergy to certain food or drug. • No major surgical operation was done. • No family history of similar condition. Patients was diagnosed as essential hypertension & on anti hypertensive drug 2 years duration Plendil (felodipine) tab. 5mg once daily.

5. Buffy hands with hand joint swelling

6. Urine analysis. Complete blood picture. Renal function tests. Liver function tests. Serum cholesterol & serum Triglyceride. Serum Na & K. Latex Rheumatoid factor. On December The following investigation were asked for:

12. Rf positive (286) n. up to 15 IU/ml. Urea 69 mg/dl (N. 10-50). CREATININE 1.67 mg/dl (N. up to 0.7-1,2 in males).

13. RHEUMATOID ARTHRITIS The case was diagnosed as :

14. Patient started treatment in the form of : • Antihypertensive drugs. • Solupred(prednisolone) tab. 5mg twice daily. • MTX sc injection 7.5mg once weekly • Folic acid tablets 500 microgm once weekly • NSAID (Delofenac sodium 100 mg tablets) once daily • Gastroprotactive( omeprazole 40 mg tablet) once daily

15. In response to Rx Blood pressure remain uncontrolled & antihypertensive drug was changed more than time ( last one was: Plendil (felodipine) tab. 5mg once daily). CBC, renal functions, liver functions and RF were checked twice in the following 4 months.

16. On February • Lab investigation reviled • UREA 82 mg/dl. • CREATININE 1.17 mg/dl. • SGOT & SGPT were elevated markedly 65 IU 78 IU MTX use was palmed as a cause for the elevated liver enzymes Nevertheless Clinical re-evaluation of the patient showed the following new signs

17. Purpuric rash on the lower legs

18. Purpuric lesions ischemic lesions

19. Leg rash typical of small vessel vacuities

20. On February To uncover the etiology of liver enzymes, HCV was assessed • HCV anti bodies : positive. • That was followed by assessment of Cryoglobulinemia • Cryoglobulin : Positive.

21. In view of the new data more lab. Investigations were requested to revile : • latex for RF : positive (431 , normal up to 15 IU/ml ). • Anti-CCP : negative • HCV anti bodies : positive. • Cryoglobulin : Positive. • Serum albumin: 3.4 (normal 3.5-5.4).

22. Patient`s positive data are: • Hypertension of 5 months duration. • Impaired renal functions. • HCV Ab positive. • RF positive. • Anti CCP negative • Cryoglobulin positive. • With clinical manifestations of peripheral vasculitis

23. Mixed cryoglobulinemia Provisional diagnosis:

25. Mixed cryoglobulinemia (MC) • Cryoglobulins are immunoglobulin complex that precipitate in the cold and dissolve on rewarming. Mixed cryoglobulinemia: cryoglobulin that contains both a polyclonal IGG and a monoclonal IGM rheumatoid factor directed against the IGG. since the identification of HCV in 1989, it has been recognized as the cause of >90% of MC.

26. In Mixed cryoglobulinemia (MC) Systemic vasculitis affecting small- and medium-sized arteries and veins. Characterized by the deposition of immune complexes containing rheumatoid factor (RF), IGG, HCV RNA, and complement on endothelial surfaces. In HCV MC blood tests show elevated serum RF, positive cryoglobulins and low complement (particularly C4).

29. MC clinical manifestations: • Many MC patients are asymptomatic • MC primarily affects the small and medium sized vessels of the skin, kidneys, and peripheral nerves. skin involvement; Purpura, primarily of the lower legs, occurs in more than 90% of patients with symptomatic HCV MC. Purpuric lesions may occasionally progress to chronic ulcers and frank gangrene.

30. Kidney involvement; • Present in approximately 20 % of patients at the time of diagnosis and occurs in 35 to 60% of patients with HCV MC. • New onset HTN can be seen in 80% of cases. • May include hematuria, nephritic to nephrotic range proteinuria, and variable progression to chronic renal insufficiency. • Glomerular disease may manifest acutely as oliguric acute renal failure in 5% of cases.

31. Peripheral nerves • Peripheral neuropathy ; sensory predominates over motor involvement  typical presentation of axonal sensory neuropathy . • Pain & paresthesia for years before motor deficits development. • Mononeuritis multiplex may also occur.

32. Treatment: • The most effective treatment for HCV MC iseradication of the underlying HCV infection. • Interferon α in combination with Ribavirin(RBV). • For patients unable to tolerate antivirals, or for those with severe renal disease, or for those who have failed to reach sustained virological response after antiviral therapy, symptomatic treatment of vasculitis with plasmapheresis, rituximab, or prednisone should be considered.

33. And the treatment was as the following; • Antihypertensive drugs. • Solupred(prednisolone) tab. 5mg twice daily. • Endoxan(cyclophosphamide) tab. 50mg once daily. As the blood pressure was still high antihypertensive drug was changed more than once ( last one is: Plendil (felodipine) tab. 5mg once daily).

34. Causes of positive RF Number of conditions beside RA (70 to 80% cases of rheumatoid arthritis) are associated with the presence of rheumatoid factor. • Autoimmune Disease: scleroderma, systemic lupus erythematosus, adult Still's disease, dermatomyositis, sarcoidosis and Sjogren's syndrome. • Infection: HIV/AIDS, hepatitis and mononucleosis, along with other viruses, endocarditis, tuberculosis and some other bacteria and parasites.

35. Cancer: leukemia and multiple myeloma. • Other Diseases: Chronic liver and lung diseases, nephroticsyndrome and cryoglobulinemia.