Scleroderma - Reset My Life

05/12/2025

🫄Why Pregnancy Is *Not Recommended* When Is Active

places major stress on the heart, lungs, kidneys, and immune system.
For women with active systemic sclerosis, this can greatly worsen the disease and increase danger for both mother and baby.

1) High risk of Scleroderma Renal Crisis (SRC)

SRC is one of the most life-threatening complications of SSc.
* Pregnancy increases blood pressure and vascular stress → can trigger SRC.
* Treatment for SRC (ACE inhibitors such as captopril) is **teratogenic** and can cause fetal death or renal failure.
* This means the mother may need life-saving medication that endangers the fetus.

📌 This combination makes active SSc pregnancy extremely dangerous.

2) Severe risks for the lungs — ILD & pulmonary hypertension (PAH)**
Women with ongoing ILD or pulmonary arterial hypertension face:

* High maternal mortality (PAH mortality in pregnancy reported up to *25–50%**).
* Risk of heart failure, respiratory failure, and preterm delivery.
* Pregnancy increases blood volume by 30–50%, worsening PAH and fibrotic lung disease.

📌 Most experts advise women with PAH to avoid pregnancy entirely.

3) Disease flare during pregnancy
If scleroderma is active, pregnancy can worsen :

* skin fibrosis
* Raynaud’s phenomenon
* digital ulcers
* internal organ involvement

Active disease often requires medications that cannot be used during pregnancy, such as :

* Mycophenolate mofetil (MMF)
* Methotrexate
* Cyclophosphamide

Thus, treatment becomes limited and unsafe for the baby.

4) High risk for the fetus
Poor blood flow and maternal organ involvement increase the risk of :

* intrauterine growth restriction (IUGR)
* miscarriage
* preterm birth
* stillbirth
* low birth weight

5) Pregnancy may mask early signs of serious complications
Typical pregnancy symptoms (swelling, fatigue, high blood pressure) can mimic :

* early renal crisis
* cardiac involvement
* pulmonary hypertension exacerbation

This may delay diagnosis and reduce survival.

✔️ Consensus Medical Recommendation
Most guidelines advise:

Only attempt pregnancy when Scleroderma is stable for at least 12–24 months and there is no progressive lung, kidney, or heart involvement.

📚 Reference (Peer-Reviewed & Guideline-Based)
1. EULAR Recommendations for Women’s Health and Pregnancy in Rheumatic Diseases (2023).
– Includes detailed guidance for Scleroderma and pregnancy safety.

2. Steen VD, Medsger TA. Systemic sclerosis and pregnancy. Rheum Dis Clin North Am. 2007.
– Classic review on maternal and fetal risks in SSc.

3. Chung L. Scleroderma renal crisis: review and recent updates. Curr Opin Rheumatol. 2013.
– Explains danger of SRC and medication limitations during pregnancy.

4. Barnes J, et al. PAH in pregnancy: maternal mortality remains high. Eur Respir Rev. 2020.
– Documents extremely high mortality for pregnant PAH patients.

5. Pattison J, et al. Outcome of pregnancy in systemic sclerosis. J Rheumatol. 1998.
– Shows increased risk of miscarriage, IUGR, and preterm birth.

6. American College of Rheumatology (ACR) Guideline on Reproductive Health in Rheumatic Diseases (2020).
– Advises pregnancy only when SSc is stable and organ involvement minimal.

03/12/2025

Scleroderma Renal Crisis (SRC)
A life-threatening emergency in systemic sclerosis

What is SRC ?
Scleroderma Renal Crisis is a sudden and severe complication of systemic sclerosis, characterized by:

Abrupt onset of very high blood pressure**, and
Rapid deterioration of kidney function**
It occurs most commonly in **diffuse cutaneous systemic sclerosis (dcSSc).**

Key Symptoms
* Sudden **severe hypertension**
* **Headache**, blurred vision
* **Shortness of breath**
* Reduced urine output
* Confusion or seizures (in severe cases)

Who Is at Highest Risk?
* Patients with **diffuse-type scleroderma**
* Rapid skin thickening within the first 3–5 years
* **High-dose steroids** (≥15–20 mg/day prednisone)
* Protein in urine or rising blood pressure
* Positive **anti–RNA polymerase III** antibody

How Is It Treated? (Very Time-Sensitive)
1) Immediate blood pressure control
ACE inhibitors** are the first-line treatment
Captopril** is preferred because it works quickly and can be adjusted rapidly

2) Close monitoring of blood pressure and kidney function
* BP must be lowered gradually but promptly

3) Dialysis if needed
* Some patients require temporary dialysis
* More than **50% can recover kidney function** after months of ACE inhibitor therapy

Warning Signs—Seek Emergency Care
* Blood pressure **≥150/85 mmHg** unexpectedly
* Rapid decrease in urine output
* Severe headache with visual changes

Prevention Tips
* Check blood pressure **daily**
* Avoid high-dose steroids unless absolutely necessary
* Routine kidney and urine tests
* Report any sudden blood pressure rise immediately

References
1. EULAR Recommendations for Systemic Sclerosis Management
2. American College of Rheumatology (ACR): Scleroderma Renal Crisis Guidance
3. Denton CP & Khanna D. *Systemic Sclerosis.* Lancet. 2017.
4. Teixeira et al. *Scleroderma renal crisis: risk factors and treatment.* Curr Opin Rheumatol.

03/12/2025

🫁Breathing Exercises for Lung Health

1) Diaphragmatic Breathing (Abdominal Breathing)**
Benefits :
* Strengthens the diaphragm
* Reduces work of breathing
* Improves oxygen exchange

How to do :
1. Sit or lie down comfortably.
2. Place one hand on your abdomen.
3. Inhale slowly and let your belly rise.
4. Exhale and allow your belly to fall.
5. Practice 5–10 minutes, 2–3 times daily.

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2) Pursed-Lip Breathing
Benefits :
* Keeps the airways open longer
* Reduces breathlessness
* Improves clearance of carbon dioxide

How to do :
1. Inhale through your nose for 2 seconds.
2. Purse your lips as if blowing out air.
3. Exhale slowly for 4–6 seconds.

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3) Box Breathing (4–4–4–4 Method)
Benefits :
* Improves lung expansion
* Helps regulate breathing
* Reduces anxiety and breathlessness

How to do :
1. Inhale for 4 seconds.
2. Hold for 4 seconds.
3. Exhale for 4 seconds.
4. Hold again for 4 seconds.

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4) Segmental Breathing
Benefits :
* Enhances lung expansion in specific areas (e.g., lower lungs)
* Useful for people with chest wall tightness or fibrosis
* Helps improve ventilation

How to do :
1. Place your hands over your lower ribs.
2. Inhale deeply and try to expand the area under your hands.
3. Exhale slowly and allow the ribs to fall.

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5) Controlled Huff Coughing
Benefits :
* Helps clear mucus from the lungs
* Reduces infection risk
* Improves airflow to deeper parts of the lungs

How to do :
1. Take a deep breath in.
2. Exhale forcefully with an open mouth, saying “huff.”
3. Repeat 2–3 times.

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6) Inspiratory Muscle Training (IMT)**
Benefits :
* Strengthens the muscles used for breathing
* Improves endurance and exercise capacity
* Helpful for patients with interstitial lung disease (including Scleroderma ILD)

How to do :
* Use a threshold IMT device.
* Train for 15–20 minutes daily.

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Who Should Practice These Exercises?
✔ People with chronic lung diseases (ILD, pulmonary fibrosis, COPD, asthma)
✔ Scleroderma patients wanting to support lung function
✔ Anyone wanting to improve lung health
❗ Stop immediately if you experience chest pain, dizziness, or severe shortness of breath.

Evidence :
Recommended by the American Thoracic Society (ATS) and European Respiratory Society (ERS) in pulmonary rehabilitation guidelines.
ATS/ERS Statement on Pulmonary Rehabilitation, *Am J Respir Crit Care Med, 2013.
Systematic reviews confirm increased inspiratory strength and function.
Beaumont M. et al., *Respiratory Medicine*, 2020
Effective airway-clearance technique used in pulmonary rehab.
McCool & Rosen, *N Engl J Med*, 2006.
Commonly recommended for improving chest mobility in rehabilitation.
Kisner & Colby, *Therapeutic Exercise*, 2017.
Proven to reduce dyspnea and improve oxygenation in chronic lung diseases.
Reference : Gosselink R., *Thorax*, 2003.

02/12/2025

30/11/2025

28/11/2025

✍️How to Avoid (or Slow) in ?

–associated interstitial lung disease (SSc-ILD) is one of the most serious complications of scleroderma. While it cannot be fully prevented, evidence shows that early detection + early treatment + lifestyle modification can significantly slow or halt progression.

✅ 1. Get Early Screening & Regular Monitoring
Early fibrosis can be silent. Screening should begin at diagnosis.
Recommended tests

High-Resolution CT (HRCT) scan** – best tool to detect early fibrosis
Pulmonary Function Tests (PFTs)** every **3–6 months** (FVC, DLCO)

Why it matters?
Early diagnosis improves outcomes and allows early treatment.
* Denton & Khanna. Lancet. 2017. “Systemic sclerosis.”
* Hoffmann-Vold et al. Ann Rheum Dis. 2019 – Guidelines emphasize HRCT + PFT monitoring.

✅ 2. Start Evidence-Based Medications Early
These improve or slow fibrosis in scleroderma:

(1) Mycophenolate mofetil (MMF)
* First-line therapy for SSc-ILD.
* Effective in stabilizing or improving lung function.

(2) Cyclophosphamide
* Effective for ILD but more side effects.
* For severe or rapidly progressive disease.

(3) Nintedanib (Ofev)
* The only FDA-approved anti-fibrotic for SSc-ILD.
* Slows the rate of lung fibrosis progressio

(4) Tocilizumab
* Helps in early diffuse cutaneous SSc + ILD stabilization.

✅ 3. Control GERD to Protect the Lungs
Micro-aspiration from acid reflux may worsen fibrosis.
Effective methods:
* Proton Pump Inhibitors (PPIs)
* Elevate head of bed
* Avoid late-night eating

✅ 4. Avoid Lung Irritants
* Don't smoke (any form)
* Avoid chemical fumes, dust, silica
* Prevent indoor air pollution

✅ 5. Vaccinations
Respiratory infections accelerate fibrosis.
Recommended:
* Influenza
* Pneumococcal
* COVID-19 booster (if applicable)

✅ 6. Exercise & Pulmonary Rehabilitation
Moderate exercise improves lung capacity and reduces decline.

✅ 7. Anti-inflammatory Diet & Lifestyle
While not a cure, they reduce oxidative stress:
* High-fiber fruits & vegetables
* Omega-3 sources (fish, chia, walnuts)
* Avoid processed foods & high sugar
* Manage stress
* Adequate sleep

📚Reference :
Herrick et al., Best Practice & Research Clinical Rheumatology, 2021 – Lifestyle factors influence SSc outcomes.
Georges et al., Clin Exp Rheumatol, 2020 – Exercise improves function and quality of life in SSc-ILD.
EULAR Recommendations, 2023 – Strongly encourage vaccination in SSc.
American Thoracic Society (ATS) ILD guidelines, 2020
Strand et al., Curr Opin Rheumatol, 2019 – GERD management is essential for SSc-ILD patients.
SENSCIS Trial, Distler et al., NEJM, 2019.
Scleroderma Lung Study I, Tashkin et al., NEJM, 2006.
SSced trial*, Khanna et al., Lancet. 2020.

Photo : Radiology Materclass

28/11/2025

Shout out to my newest followers! Excited to have you onboard! Deb Engler, Marivic Mata, Mischelle Nielson

26/11/2025

What is the most likely diagnosis❓

A. Psoriasis
B. Dermatomyositis
C. SLE
D. Scleroderma

Cr. nervemed

23/11/2025

Scleroderma can leave marks on the skin that tell a story of strength, endurance, and daily battle. For many warriors, the disease becomes visible through skin tightening, discoloration, swelling, or changes in facial features and hands. These outward signs are more than physical differences—they are reminders of what the body is enduring. Visible scleroderma can draw attention, questions, or even judgment, making the emotional weight just as heavy as the physical symptoms.

The visible effects of scleroderma are diverse and deeply personal. Some warriors experience hardened skin that affects movement, while others face noticeable swelling or changes in texture that can limit flexibility and comfort. These physical changes can make simple tasks more difficult, from bending fingers to walking comfortably. Though the signs are seen on the outside, the battle is still happening within—each visible mark representing resilience, adaptation, and courage.

Because visible scleroderma is so noticeable, understanding and empathy become even more important. Warriors may struggle with confidence, self-image, or unwanted attention from people who do not know the condition. By raising awareness, we remind the world that these changes do not define a person—they reveal a fighter who continues to push forward. When we recognize the visible signs with compassion instead of judgment, we help create a community where every warrior feels respected, supported, and seen.