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138 Autoimmune Adrenal Insufficiency Antedates the Diagnosis of SLE, Does It Really Matter June 2011 insufficiency, as proved by hyper-pigmentation, hypotension, extreme fatigue and low serum cortisol level (203 nmol/l) which failed to rise one hour after intravenous injection of 0.25 mg co-syntropin two years prior to her presentation with features of SLE (fulfilling the ARC criteria) that manifested as malar rash, discoid rash, photosensitivity, painless oral ulcers, arthritis, hemolytic anemia and renal involvement. As SLE is a multi system disorder, many of the clinical features are common with adrenal insufficiency, Among 20 reported cases with positive anticardiolipin antibodies with adrenal failure, only four cases developed features of SLE [5,9] . In our case, the autoimmune adrenal insufficiency antedated the clinical diagnosis of SLE. This could be due to the fact that, the immune destruction of the adrenals started earlier than other tissue in the body or long term steroid therapy for autoimmune adrenal insufficiency may have masked the clinical presentation of SLE. Another explanation may be that many of the non-specific clinical features of SLE are common with adrenal insufficiency. This per se can create a great confusion in clinical diagnosis in the beginning, but with close and careful follow-up, definite diagnosis can be made easily, as other symptoms appear with time. This is what happened with our patient. Moreover, it is very unlikely that these two clinical conditions are not related pathologically and only co-existed in the same patient. The etiology of hypoadrenalism in SLE is unknown, but proposed mechanisms may be adrenal vascular thrombosis and infarction, hemorrhage due to abnormal coagulation, vasculitis and a direct organ specific autoimmune insult [2,4,6,8] . Early reports of the association suggested the presence of antiphospholipid antibodies (APLA) in these patients, and hence the adrenal failure has been related to their presence [3,4,6] but APLA were not found in our patient despite being diagnosed with SLE. In SLE, adrenal involvement may be the first clinical manifestation of this syndrome, whereas a few patients may have history of adrenal insufficiency in the past [2,8] . In some cases of adrenal damage due to hemorrhage, incomplete destruction of adrenal cortex may leave enough residual function to prevent acute adrenal crisis, with later development of chronic adrenal insufficiency [2] . The association between SLE and adrenal insufficiency has been described earlier in the literature. In all reported cases, the diagnosis of adrenal insufficiency was made after the diagnosis of SLE [5,6] and in some cases, both conditions have been described simultaneously [6,9-11] . We describe for the first time in the literature, autoimmune adrenal insufficiency antedating the diagnosis of SLE with negative APLA. This proves the hypothesis of the presence of other pathological mechanisms rather than APLA, in this association. CONCLUSION This case suggests the need for increased suspicion of SLE in patient with adrenal insufficiency and systemic complaints. This clinical suspicion should be high when patient with adrenal insufficiency present with arthalagia, skin rash, anemia and raised ESR. To confirm the diagnosis, ANA and anti-ds DNA should be done, but APLA may not be found in these patients. Therefore, we advise careful follow up of such patients since they may evolve into connective tissue disease. REFERENCES 1. Laureti S, Vecchi L, Santeusanio F, Falorni A. Is the prevelance of Addison’s disease underestimated J Clin Endocrinol Metab 1999; 84:1762. 2. Peterson P, Uibo R, Krohn KJ. Adrenal autoimmunity: results and developments. Trends Endocrinol Metab 2000; 11:285-290. 3. Asherson RA, Hughes GR. Hypoadrenalism, Addison’s disease and antiphospholipid antibodies. J Rheumatol 1991; 18:1-3. 4. Alarcon-segovia D. Pathogenetic potential of antiphospholipid antibodies. J Rheumatol 1988; 15:890- 893. 5. Eichner HL, Schambelan M, Biglieri EG. Systemic lupus erythematosus with adrenal insufficiency. Am J Med 1973; 55:700-705. 6. Koren S, Hanly JG. Adrenal failure in systemic lupus erythematosus. J Rheumatol 1997; 24:1410-1412. 7. Betterle C, Volpato M, Rees Smith B, et al. Adrenal cortex and steroid 21-hydroxylase autoantibodies in children with organ-specific autoimmune diseases: markers of high progression to clinical Addison’s disease. J Clin Endocrinol Metab 1997; 82:939-942. 8. Lie JT. Vasculopathy in the antiphospholipid syndrome: thrombosis or vasculitis, or both J Rheumatol 1989: 16:713-715. 9. Zhang ZL, Wang Y, Zhou W, Hao YJ. Addison’s disease secondary to connective tissue diseases: a report of six cases. Rheumatol Int 2009; 29:647-650. 10. Asherson RA, Cervera R. Unusual manifestations of the antiphopholipid syndrome. Clin Rev Allergy Immunol 2003; 25:61-78. 11. Espinosa G, Cervera R, Font J, Asherson RA. Adrenal involvement in the antiphospholipid syndrome. Lupus
June 2011 KUWAIT MEDICAL JOURNAL 139 Case Report Macroinvasive Papillary Thyroid Carcinoma Presenting as Internal Jugular Vein Tumor Thrombus Ayman Farouk Elezeby 1 , Ibrahim Alenezi 1 , Medhat Mohamed Saber Elsherbiny 2 1 Department of Surgery, Al-Jahra Hospital, Kuwait 2 Department of Radiology, Al-Jahra Hospital, Kuwait ABSTRACT Kuwait Medical Journal 2011; 43 (2): 139-142 Papillary thyroid carcinoma with massive invasion into the great veins of the neck and mediastinum has rarely been reported and is thought to have a poor prognosis. But multimodal therapeutic approach comprising of surgery, radioiodine and external beam radiotherapy may give best results for patients in whom thyroid cancer is occluding the great veins. Here we report successful management of a case of papillary thyroid carcinoma with extensive invasion into the left internal jugular vein. KEY WORDS: metastatic thyroid carcinoma, thyroid neoplasm INTRODUCTION Microscopic vascular invasion is well recognized in thyroid cancer particularly in the follicular and poorly differentiated histological types [1,2] . However massive invasion of papillary thyroid carcinoma into the great veins of the neck is rare. Management of these patients is challenging as they typically present with advanced and rapidly progressive disease. We describe the clinicopathological finding and surgical management of a case of papillary thyroid carcinoma with extensive invasion into the left internal jugular vein (IJV). CASE REPORT A 47-year-old Philippino lady, not known to have any previous medical problems, presented with a painless firm swelling in the left side of the neck of six months duration and past history of right thyroid lobectomy two years ago in an overseas hospital. There was no operative or pathological report available. On physical examination there was a tender swelling, 2 x 2 cm, deep to lower third of left sternocledomastoid muscle and multiple painless firm mobile discrete upper and lower deep cervical lymph nodes. There was no facial edema or any dilated veins over chest wall. Patient was clinically and biochemically euthyroid. Color Doppler sonography showed presence of multiple hypoechoic hypovascular nodules in left thyroid lobe. The largest of them measured 15 mm, exhibiting a homogeneous hypoechoic texture with clear border and multiple enlarged cervical lymph nodes along the left jugular chain. The left IJV was seen totally occluded above the subclavian vein being filled with large expanding soft tissue thrombus that exhibited color flow signals within. Computed tomography (CT) scan confirmed the tumoral thrombus obstructing the IJV with dilated collaterals at the surface of the left lobe of the thyroid (Fig. 1, 2). Ultrasound guided fine-needle aspiration cytology (FNAC) of left thyroid lobe and left cervical lymph nodes revealed feature of thyroid papillary carcinoma with cervical lymph node metastasis. At operation we found tumor in the left thyroid lobe and the superior thyroid vein was dilated, tortuous and full of malignant mass. The left IJV also had a pedunculated tumor mass blocking the vein. It was about 4 cm long and partly attached to the wall of the vein (Fig. 3, 4). Left thyroid lobectomy and modified block dissection involving levels 2, 3, 4, 5A, 6 with excision of IJV from the level of hyoid bone to the lower end of internal jugular vein was performed. The left accessory nerve and sternocledomastoid muscle were preserved. The postoperative course was uneventful. Pathologic examination showed multifocal papillary carcinoma, 0.1 to 1.3 cm, with no extrathyroid extension (Fig. 5). There was metastasis Address correspondence to: Ayman Farouk Elezeby, MSc MD MRCS, Department of Surgery, Al Jahra Hospital, Kuwait. PO Box 305 Al Jahra, Tel:00965 97898295, E-mail: aymanezaby@yahoo.com
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