1 / 32

Interesting Case

. 44 year old maleDriverIdiki No co-morbidities. Complaints of : . Bilateral periorbital swelling

wheeler
Download Presentation

Interesting Case

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript

    1. Interesting Case Sunny Thomas 747738 01/04/09

    2. 44 year old male Driver Idiki No co-morbidities

    3. Complaints of : Bilateral periorbital swelling & swelling in bilateral parotid & sub mandibular region since 5 yrs Sudden onset of blurring of vision in Right Eye followed by the Left Eye – 22 days back.

    4. Bilateral periorbital swelling & swellings in parotid and submandibular regions since 5 years Insidious in onset Gradually progressive Not associated with pain, tenderness, fever h/o dryness of eyes + No h/o dryness of mouth No h/o dysphagia No h/o joint pains, oral ulcers

    6. Sudden onset of blurring of vision in Right eye followed by Left eye – 22 days back Only perception of light + in Right eye No h/o headache, vomiting, scotomas, flashes of light No h/o trauma Taken to ARAVIND EYE HOSPITAL, Madurai Diagnosed to have Right eye CRVO and was referred here

    7. Past History Evaluated in Kottayam Medical College in october 2005 Schimmer test :+ ESR : 115 mm/1st hr ANA Screen : negative Serum Electrophoresis : elevated gamma globulin Cranio orbital CT : Enlarged lacrimal gland with altered attenuation of surrounding fat tissue – probably inflammatory origin, pseudotumor polyps in all sinuses

    8. evaluated in Aravind Eye Hospital in february 2006 : Schimmer test + Elevated ESR Lacrimal gland biopsy : nonspecific chronic dacrocystadenitis Evaluated in Kollencherry Medical College in March 2008 : Lymphnode biopsy : reactive hyperplasia ANA profile : negative

    9. On examination : Conscious and coherent No pallor, icterus, cyanosis, clubbing, pedal edema Lymphadenopathy + : cervical, axillary, inguinal non matted non tender Swelling around both the eyes : non tender Rt eye : CRVO, later vitreal haemorrhage Lt eye : CRVO

    10. PULSE : 74 /min, regular BP : 128/80 mm Hg TEMP : 98.6 F RR : 14 /min, regular

    11. RS : B/L Normal vesicular breath sounds no added sounds CVS : S1+, S2 +, No murmurs P/A : soft non tender hepatomegaly+ NS : HMF : Normal No Cranial nerve palsies No sensory motor deficit No signs of cerebellar dysfunction DTR : B/L + Plantar : b/l flexor

    12. Left eye

    13. Right eye

    14. Left eye

    15. Right eye

    16. Right eye

    17. Right eye

    18. Right eye

    19. CBC : TC : 4.48 K/ul N: 26% L: 59% E: 7 % Hb : 11.2 g/dl ESR : 68 mm/1st hour Urine R/E : Within normal limits LFT : A/G reversal( 2.3/5.5 ) S.Creatnine : 0.95 mg/dl RBS : 97 mg/dl

    20. ANA Profile : Negative Anti ds DNA : 14 IU/ml ( negative ) TFT : within normal limits Serum electrophoresis : M band present S.LDH : 160.9 U/L S.ACE : 82 U/L Coombs direct and indirect : negative

    21. CXR : normal ECG : normal Xray skull : no lytic lesions RA factor : > 512 IU/ml ( positive ) Mantoux : negative USG Abdomen : mild splenomegaly

    22. Peripheral blood smear : normocytic normochromic blood picture with mild rouleux formation and thrombocytopenia Bone Marrow Aspirate: normocellular marrow with trilineage maturation & megakaryocytic abnormalities representing increased platelet destruction Bone Marrow Biopsy : cellular marrow with trilineage maturation Lymph node biopsy : ( axillary ) reactive paracortical hyperplasia

    23. Anti phospholipid antibody IgG: 7.41 GPLunit/ml ( 0-9.9 ) Anti phospholipid antibodyIgM:10.18MPLunit/ml (0 – 9.9 ) Gammaglobulin IgE : 269.8 IU/ml ( 0-100 ) Gammaglobulin IgG : 4157.3 mg/dl ( 700-1600 ) Gammaglobulin IgM : 140.5 mg/dl (40-230 ) Gammaglobulin IgA : 210.4 mg/dl ( 70 -400 )

    24. Diagnosis MIKULITCZ SYNDROME PRESENTING AS BILATERAL NON ISCHEMIC CRVO

    25. Mikulicz's disease Mikulicz's disease (MD) has been considered part of primary Sjögren's syndrome (SS) since Morgan's report in 1953. MD represents a unique condition involving enlargement of the lacrimal and salivary glands, as is also seen in SS MD is characterized by few autoimmune reaction and its good responsiveness to glucocorticoid. Recent reports have shown that the frequency of apoptosis in glands of MD patients is lower when compared with SS. This phenomenon reflects the histologically reversible gland secretion in MD.

    26. Sialography in patients with mikulicz disease did not show APPLE TREE SIGN Apple tree sign represents contrast medium filling out from salivary acini and ducts of severely degenerated or destroyed glands in sjogrens syndrome. The absence of this sign indicates a lack of glandular destruction in Mikulicz disease.

    27. . Elevated IgG4 concentrations in the serum and prominent infiltration by plasmacytes expressing IgG4 in the lacrimal and salivary glands have also been confirmed in MD. Plasma cells expressing IgG4 are also detected in lymph nodes and bone marrow. MD may be a IgG4 related systemic disease, rather than a lacrimal and salivary gland disease Patients of Mikulicz disease are anti SS-A & antiSS-B antibodies negative and they have elevated IgG4 concentrations in serum.

    28. Hence, Mikulicz disease is quite different from Sjogerens syndrome both clinically and histopathologically .

    29. It is also known as : Dacryosialoadenopathia Dacryosialoadenopathy Mikulicz- Radecki Syndrome Mikulicz-Sjogren Syndrome Von Mikulicz Syndrome

    30. Although always benign , it can occur in association with another underlying disorder such as : Tuberculosis Lymphoma Sarcoidosis Leukemia Syphilis Hodgkin’s disease Lymphosarcoma Sjogrens syndrome SLE

    31. The exact cause of Mikulicz syndrome is not known . Some scientists believe that it should be considered a form of sjogrens syndrome and while some don’t !

    32. Other causes of elevated IgG4 Pemphigus vulgaris Pemphigus foliaceus Autoimmune pancreatitis Tubulointerstitial nephritis Riedel’s thyroiditis

    33. Thank you

More Related