19/03/2024
কেইস ফাইলস
ডা: মো: আ: মোতালিব
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মি, সিরাজ, ৪৩ বছর। কার্ডিওলজিতে ভর্তি ছিলেন। শ্বাসকষ্ট। মেডিসিনে ট্রান্সফার করা হয়েছে। দেখছেন রেসপিরেটরী স্পেশালিষ্ট।
স্বাসকষ্ট কমছেনা। রাউন্ডের সময় রোগিটিকে দেখলাম। মি, সিরাজ বললেন, স্যার রানের চামড়া শক্ত হয়ে যাচ্ছে।কাশি, পায়ে পানি, খাবার গিলতে সমস্যা।
দেখলাম, আসলেই তাই। Leg দিকে তাকালাম, চামড়া, Hard with mild edema. হাত দেখলাম, আংগুলের চামড়া Tight & thick, giving the appearance of slcerodactyly
বুঝতে বাকি রইলোনা যে, Its a clear case of systemic sclerosis (SSc).
So, why breathlessness?
Possibilities are-...Pulmonary fibrosis from lung involvement by SSc...Pulmonary hypertension...Scleroderma renal crisis...Flush pulmonary edema...Heart failure from 1. Cor-pulmone due to pulmonary fibrosis. 2. CAD due to endothelial dysfunction. 3 Cardiomyopathy. 4. Arrythmia from endocardial fibrosis.
In SSc, by autoimmunity, there develops endothelial dysfunction. This leads to failure of release of NO but excess action o ET (Endothelin). And this causes vasoconstriction.
In renal vascular bed, vasoconstriction causes activation of RAAS ( renin- angiotensin aldosterone system) which leads to salt & water retention . So hypertension develops, what we call scleroderma renal crisis. Renal bed also causes break down of RBC while flowing through microcirculation & develops MAHA ( Micro- angiopathic hemolytic anemia).
On the other hand, there is endothelial leakage in pulmonary capillary causing sudden development of SOB (shortness of breathing), we call it flush pulmonary edema, provided that there is no evidence of heart failure ( Normal pulmonary wedge pressure & normal LV EF% .
For Corpulmonale, there will be evidence of pulmonary hypertension by the presence of any/all of the three-
1. Left parasternal heave,
2. Palpable & loud P2,
3.Tricuspid regurgitation (PSM in LLSE with pulsatile liver)
along with Evidence of Pulmonary fibrosis by fine bibasal crepitation in lungs,
with/without right heart failure
1.Evidenced by raised, pulsatile JVP,
2.Edema +/-ascites
LVF/ cardiomyopathy from myocardial fibrosis & autoimmune myositis would be evidenced by
1. Low BP,
2. Weak rapid pulse,
3. Cold clammy periphery
4. Shifted apex beat with
5. Bibasal lung creps
Skin tightening & thickening results from autoimmune activation of firbrobast, PDGF, that causes deposition of more & more deposition of collagen fibre on skin & also results in arteriolar sub-intimal thickening (fibro-proliterative vasculopathy), both of these leading to development of thick skin and raynalds phenomen from endothelial dysfunction hands arteriolar system causing color change of figers on exposure to cold.
There is also dysphagia from esophageal involvements, other parts of gut may involve causing malabsoetion syndrome with loss villi & micro- villi, even bacterial overgrowth syndrome.
What a miracle arises from over- activation od Fibrobast and release of PDGF & endothelial dysfunction.
Mostly it is due to atoimmune problem. With delayed & prolonged apoptosis leads to exposure of nuclear material to immune system & ultimate development of auto- antibodies eg ANA, ant Scl-70, anti-CM.