Sjogrens Syndrome and Scleroderma MCQs

Atopic Keratoconjunctivitis: Demographics: Generally seen in young adults. Associated Atopic Diseases: Often associated with a history of other atopic diseases such as: Asthma Allergic rhinitis Atopic eczematoid dermatitis in the patient or their family. Disease Characteristics: Responsible for an extremely severe and chronic form of ocular surface disorder. The disease spectrum varies in range of severity: Ranging from mild cases to chronic and persistent inflammation. Comparison to VKC (Vernal Keratoconjunctivitis): Similar in nature to VKC but tends to be more: Severe Chronic Extends into adulthood. Reference: Parson's Diseases of the Eye-Edition 23-Chap 11-Diseases of the Conjunctiva

The cause of chronic GI blood loss in a patient with SSC is Gastric Antral Vascular Ectasia. The endoscopic appearance is Water melon stomach. Reference : Harrisons Principles of Internal Medicine, 21 st ed, Ch: 360, Pg: 2781

Scleroderma can cause folate deficiency due to malabsorption. However, in this case, the folate levels are normal and there is B12 deficiency. B12 deficiency can occur in conditions where there is bacterial overgrowth in the small intestine (blind loop syndromes) such as jejunal diverticulosis, Crohns disease, fistulas and scleroderma. The anaerobic organisms metabolise vitamin B12 and impair absorption. When systemic sclerosis (SSc) involves the small intestine, normal peristaltic movements are lost and motility is impaired leading to stasis and dilatation. Reference: Harrison’s Principles of Internal Medicine 21st edition Chapter:360 Page number:2781

Calcinosis cutis is more seen with long standing systemic sclerosis, especially the anti centromere positive limited SSc. The serum calcium and phosphate levels are normal and the deposits are composed of calcium hydroxyapatite. The calcifications usually occur in finger pads, palms, extensor aspects of forearm. Large deposits can cause local nerve compression. Paraspinal sheet calcification can be seen with neurological complications. The lesions can ulcerate the overlying skin too. Reference : Harrison's principles of Internal Medicine, 21 st ed, Ch: 360, Pg: 2779

Three cardinal pathomechanistic processes underlie the protean clinical manifestations and pathologic changes of SSc: diffuse microangiopathy, inflammation and autoimmunity, and visceral and vascular fibrosis affecting multiple organs. In general, autoimmunity and reversible vascular reactivity are early features of SSc, whereas fibrosis and atrophy occur later in the disease. Diffuse Microangiopathy is the first and primary pathogenic event and is responsible for most of the clinical symptoms, including, telangiectasis, Raynaud's Phenomenon, digital ulcers, renal crisis, myocardial involvement and PAH. Reference: Harrison's Principles of Internal Medicine 21st edition Chapter:360 Page number:2773

Anti-Ribosomal antibodies are not typically associated with Scleroderma as their phenotype. Anti-Pm-Scl antibodies : These are often found in patients with overlap syndromes involving features of both Scleroderma and Polymyositis. Anti-Ku antibodies : These are associated with some cases of Scleroderma, particularly the limited cutaneous form. Anti-U1 RNP antibodies : These are more commonly associated with Mixed Connective Tissue Disease (MCTD), which can have overlapping features of Scleroderma among other autoimmune conditions. Anti-Ribosomal antibodies : These antibodies are primarily associated with systemic lupus erythematosus (SLE) and are not a typical feature of Scleroderma. Reference: Harrison's Principles of Internal Medicine 21st edition Chapter:360 Page number:2776

CQ is being used in RA not in ARF CQ is being used in the following conditions. My RED LIP M-malaria R- RA E- Extraintestinal Amebiasis D- Discoid Lupus L- Lepra Reaction I- Infective Mononucleosis P-Photogenic reaction Reference : Harrison's Principles of Internal medicine, 21st edition Ch: 223Pg 1732

Accelerated hypertension and bilateral papilledema in a patient with Scleroderma indicates Scleroderma Renal Crisis. Accelerated hypertension There will be a rapid decline in renal function, with nephrotic range proteinuria. First line treatment is with ACE inhibitors. ARBs are given only if patient is intolerant to ACEI. Goal is to reduce BP by 20/10mmHg every 24 hours till normal. UIII RNP is a predictor of risk of renal crisis in Scleroderma Anti centromere antibody is a negative predictor. Renal biopsy shows changes of accelerated hypertension. Reference: Harrison's Principles of Internal Medicine, 21 st edition, Ch:317, Pg:2366.