Raiyyan Tahsan

Raiyyan Tahsan
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TC(1039).(05).Orthopedics Case No 117:- Myositis

Myositis — Bangla Clinical Note

Myositis মানে skeletal muscle-এর প্রদাহজনিত রোগ, যেখানে muscle fibre damage হয়ে proximal muscle weakness, muscle enzyme rise, pain/ক্লান্তি, skin rash, dysphagia, lung/cardiac involvement হতে পারে। Medical practice-এ বেশি গুরুত্বপূর্ণ group হলো Idiopathic Inflammatory Myopathy/IIM—Dermatomyositis, Polymyositis, Immune-mediated necrotizing myopathy, Antisynthetase syndrome, Inclusion body myositis। 2017 EULAR/ACR criteria IIM classification-এ muscle weakness pattern, skin signs, dysphagia, anti-Jo-1, muscle enzymes, biopsy findings ব্যবহার করে; ≥55% probability “probable IIM” এবং ≥90% “definite IIM” হিসেবে ধরা হয়।

Prescription note: নিচের drug-dose adult practice-এর educational framework। Patient-specific dose renal/hepatic function, pregnancy, infection, malignancy risk, CK trend, ILD severity, TPMT/NUDT15 status অনুযায়ী Rheumatologist/Medicine specialist supervise করবেন।

1) Definition / সংজ্ঞা

  • Myositis = muscle inflammation; clinically এর core presentation হলো symmetrical proximal muscle weakness—চেয়ার থেকে উঠতে কষ্ট, সিঁড়ি উঠতে কষ্ট, মাথার ওপর হাত তুলতে কষ্ট।
  • Dermatomyositis হলে muscle weakness-এর সাথে typical skin rash থাকে—heliotrope rash, Gottron papules/sign, shawl/V-sign rash।
  • Antisynthetase syndrome হলে myositis + ILD + arthritis + Raynaud + mechanic’s hands দেখা যায়।
  • Inclusion body myositis সাধারণত elderly male-এ slow progressive, quadriceps ও finger flexor weakness বেশি, steroid response poor।

2) Causes / কারণ — point আকারে

A. Autoimmune / idiopathic inflammatory causes

  • Dermatomyositis
    • Muscle ও skin-এর small vessel injury/complement-mediated microangiopathy।
    • Anti-Mi-2, anti-TIF1-γ, anti-NXP2, anti-MDA5 antibody associated হতে পারে।
    • Adult dermatomyositis-এ malignancy association থাকে; BSR guideline adult-onset IIM-এ cancer risk consider করতে বলেছে, বিশেষ করে older age, male sex, dysphagia, rapid onset, anti-TIF1-γ/anti-NXP2 positive হলে।
  • Polymyositis / overlap myositis
    • Endomysial inflammatory cell infiltration, CD8 T-cell mediated muscle fibre injury।
    • SLE, systemic sclerosis, mixed connective tissue disease-এর সাথে overlap হতে পারে।
  • Immune-mediated necrotizing myopathy
    • Severe CK rise, prominent necrosis with little inflammation।
    • Anti-SRP বা anti-HMGCR antibody positive হতে পারে।
    • Statin exposure-এর পর trigger হতে পারে; তবে statin বন্ধ করলেও autoimmune disease চলতে পারে।
  • Antisynthetase syndrome
    • Anti-Jo-1, PL-7, PL-12, EJ, OJ antibody।
    • ILD অনেক সময় dominant presentation।
  • Inclusion body myositis
    • Inflammatory + degenerative pathology।
    • Quadriceps wasting, finger flexor weakness, falls, dysphagia common।

B. Infective myositis

  • Viral: influenza, HIV, hepatitis, EBV, COVID-associated myalgia/myositis।
  • Bacterial: pyomyositis, commonly Staphylococcus aureus।
  • Parasitic: trichinellosis, toxoplasmosis—history অনুযায়ী।

C. Drug/toxin-induced

  • Statin, fibrate, colchicine, alcohol, cocaine, zidovudine, interferon, immune-checkpoint inhibitors।
  • Chronic steroid নিজেও steroid myopathy করতে পারে—এতে CK usually normal/low, pain কম।

D. Endocrine/metabolic

  • Hypothyroidism, hyperthyroidism, Cushing syndrome।
  • Hypokalemia, hypophosphatemia, vitamin D deficiency।
  • Rhabdomyolysis: exertion, heat illness, seizure, trauma।

3) Symptoms / Clinical features — Bangla point আকারে

Muscle symptoms

  • Proximal weakness
    • চেয়ার/নিচু জায়গা থেকে উঠতে কষ্ট।
    • সিঁড়ি উঠতে কষ্ট।
    • চুল আঁচড়ানো, কাপড় শুকাতে দেওয়া, মাথার ওপর হাত তোলা কঠিন।
  • Neck flexor weakness
    • মাথা বালিশ থেকে তুলতে কষ্ট।
  • Myalgia / muscle tenderness
    • সব রোগীতে থাকে না; inflammatory flare বা infective myositis-এ বেশি।
  • Fatigue ও weight loss
    • chronic inflammation বা malignancy-associated disease হলে বেশি।
  • Falls
    • quadriceps weakness, especially inclusion body myositis।

Skin symptoms — Dermatomyositis

  • Heliotrope rash: চোখের পাতা বেগুনি/লালচে swelling।
  • Gottron papules/sign: MCP/PIP joint-এর ওপর violaceous papule।
  • Shawl sign / V-sign: ঘাড়, upper back, chest-এ photosensitive rash।
  • Mechanic’s hands: আঙুলের পাশে rough cracked hyperkeratotic skin—antisynthetase syndrome।
  • Periungual erythema / nailfold capillary change।
  • Calcinosis: skin/subcutaneous calcium deposit—juvenile DM-এ বেশি।

Systemic symptoms

  • Dysphagia: খাবার গিলতে কষ্ট, nasal regurgitation, aspiration risk।
  • Dyspnea / dry cough: ILD সন্দেহ।
  • Arthralgia/arthritis: antisynthetase বা overlap disease।
  • Raynaud phenomenon।
  • Palpitation/chest pain: myocarditis, arrhythmia, cardiomyopathy suspicion।
  • Fever: infection, malignancy, active systemic inflammation।

4) Clinical diagnosis — specific approach

Bedside clinical diagnosis

  • History
    • Weakness onset: acute/subacute/chronic।
    • Drug history: statin, colchicine, steroid, antimalarial, alcohol, immune checkpoint inhibitor।
    • Rash, Raynaud, arthritis, cough/dyspnea, dysphagia, weight loss।
    • Cancer red flags: unexplained weight loss, bleeding, lump, anorexia, new anemia।
  • Examination
    • MRC grading or MMT-8 muscle strength scoring।
    • Proximal vs distal distribution।
    • Skin: Gottron, heliotrope, shawl/V sign, mechanic’s hand।
    • Lung: basal crackles.
    • Cardiac: pulse irregularity, signs of heart failure.
    • Swallow assessment red flag.

Differential diagnosis

  • Hypothyroid myopathy।
  • Statin-associated myopathy / rhabdomyolysis।
  • Muscular dystrophy / metabolic myopathy।
  • Myasthenia gravis—fatigable weakness, ocular/bulbar।
  • Motor neuron disease / peripheral neuropathy।
  • Polymyalgia rheumatica—pain/stiffness বেশি, true weakness কম।
  • Steroid-induced myopathy—painless proximal weakness, CK often normal।
  • Infective pyomyositis—localized pain, fever, abscess।

5) Investigations — specifically what to order

Investigationকী দেখব / কেন করব
Serum CKMost useful muscle injury marker; flare monitoring।
Aldolase, LDH, AST/ALTMuscle enzyme rise; AST/ALT liver disease ভেবে ভুল হতে পারে।
CBC, ESR/CRP, LFT, RFT, urine R/M/EBaseline disease + drug safety।
TSH, electrolytes, vitamin DMimic/endocrine myopathy exclude।
ANA, ENAOverlap connective tissue disease।
Myositis-specific antibodiesAnti-Jo-1, PL-7/PL-12, Mi-2, TIF1-γ, NXP2, MDA5, SAE, SRP, HMGCR; BSR guideline myositis autoantibody testing recommend করে, কারণ এগুলো diagnosis, phenotype, prognosis ও treatment tailoring-এ সাহায্য করে।
EMG/NCSMyopathic motor unit, fibrillation; neuropathy/MG differentiate।
MRI muscle—STIR/T2 thigh/shoulderMuscle edema, active inflammation, biopsy site selection।
Muscle biopsyDM: perifascicular atrophy; PM: endomysial inflammation; IMNM: necrosis; IBM: rimmed vacuoles।
Skin biopsyDermatomyositis rash uncertain হলে।
PFT with DLCO + HRCT chestILD screen/monitor; BSR high-risk adult IIM-এ ILD screening recommend করে।
ECG, Troponin-I, Echocardiography ± Cardiac MRIMyocarditis/arrhythmia/cardiomyopathy suspicion।
Swallow evaluationDysphagia/aspiration risk; BSR guideline dysphagia routine assessment consider করতে বলে।
Cancer screeningAge/sex appropriate screening; high-risk DM/IIM-এ CT chest-abdomen-pelvis, pelvic/testicular/breast/colon evaluation, selected tumour markers/PET-CT।

6) Complications — Bangla ব্যাখ্যা

  • Interstitial lung disease
    • Lung-এর interstitium inflamed/fibrosed হয়।
    • Dry cough, exertional dyspnea, hypoxia; anti-MDA5 disease-এ rapidly progressive ILD হতে পারে।
  • Dysphagia ও aspiration pneumonia
    • Pharyngeal/esophageal muscle weak হলে খাবার airway-তে ঢুকে pneumonia করতে পারে।
  • Respiratory muscle weakness
    • Severe disease-এ ventilatory failure হতে পারে।
  • Cardiac involvement
    • Myocarditis, arrhythmia, heart failure; chest pain/palpitation হলে urgent work-up।
  • Calcinosis
    • Skin/subcutaneous calcium deposit; pain, ulcer, infection করতে পারে।
  • Contracture, muscle atrophy, disability
    • Long untreated disease বা prolonged inactivity।
  • Malignancy-associated myositis
    • Adult dermatomyositis-এ clinically important।
  • Treatment complications
    • Steroid: diabetes, HTN, osteoporosis, infection।
    • Immunosuppressants: marrow suppression, hepatotoxicity, opportunistic infection।

7) Treatment management — clinical algorithm

BSR guideline অনুযায়ী active muscle inflammation induction-এ high-dose glucocorticoid ব্যবহার করা হয়; adult oral prednisolone 0.5–1 mg/kg/day, usually 40–60 mg/day recommended। Disease activity improve করলে সাধারণত প্রায় 6 সপ্তাহ পরে taper শুরু করা হয়; early steroid-sparing DMARD যেমন methotrexate, azathioprine, tacrolimus, cyclosporine, mycophenolate ব্যবহার করে remission maintain করা যায়। Refractory/severe disease-এ cyclophosphamide, rituximab, IVIG consider করা হয়।

Bangladesh doctors prescription-এ commonly used drug list — কেন ব্যবহার হয়

  • Prednisolone / Methylprednisolone: acute inflammation দ্রুত control করতে।
  • Methotrexate: steroid-sparing first-line DMARD; muscle ও skin disease।
  • Azathioprine: steroid-sparing; myositis + ILD/overlap disease; pregnancy-compatible option হিসেবে specialistরা consider করেন।
  • Mycophenolate mofetil: ILD বা skin/muscle refractory disease-এ বেশি ব্যবহৃত।
  • Hydroxychloroquine: dermatomyositis skin rash/photosensitive rash-এ; muscle weakness-এর primary drug নয়।
  • Tacrolimus / Cyclosporine: antisynthetase syndrome/IIM-ILD, especially refractory বা rapidly progressive ILD।
  • Cyclophosphamide: severe ILD, life-threatening organ involvement, refractory disease।
  • Rituximab: refractory myositis, autoantibody-positive disease, ILD, severe skin disease।
  • IVIG: severe/refractory dermatomyositis, dysphagia, rapidly progressive weakness, pregnancy/contraindication context; ProDERM trial-এ active dermatomyositis-এ IVIG 2 g/kg প্রতি 4 সপ্তাহে placebo-এর চেয়ে better response দেখিয়েছে।
  • Supportive: folic acid with MTX, PPI if steroid/NSAID GI risk, calcium-vitamin D ± bisphosphonate, TMP-SMX for PJP prophylaxis when high-risk immunosuppression।

8) Pharmacological drugs — generic, class, dose, duration, side effects, interaction, Bangladesh brand

8.1 Prednisolone

  1. Generic name: Prednisolone
  2. Therapeutic category: Systemic anti-inflammatory / immunosuppressant
  3. Pharmacological class: Glucocorticoid corticosteroid
  4. Why used: Myositis inflammation দ্রুত কমাতে induction therapy।
  5. Dose: Adult 0.5–1 mg/kg/day PO, usually 40–60 mg/day for active disease; 4–6 weeks পরে response অনুযায়ী taper। BSR guideline adult prednisolone 0.5–1 mg/kg/day, usually 40–60 mg recommend করে।
  6. Duration: Usually months; taper CK, strength, rash, ILD response অনুযায়ী।
  7. Side effects: Hyperglycemia, hypertension, weight gain, gastritis, mood change, insomnia, acne, infection, osteoporosis, cataract/glaucoma, adrenal suppression।
  8. Interaction: NSAID/aspirin → GI bleed risk; live vaccine avoid; antidiabetic/antihypertensive dose adjustment লাগতে পারে; CYP3A inhibitors steroid effect বাড়াতে পারে।
  9. Bangladesh available brand names with strength — Medex listed: Adam 33 5/20 mg, Cortan 2/5/10/20 mg & 5 mg/5 ml solution, Cortisol 5/10/20 mg & 5 mg/5 ml solution, Delta 5 mg, Deltacort 5 mg, Deltapred 5/10 mg, Deltasone 5/10/20 mg & 5 mg/5 ml solution, Edruson 5 mg, G-Prednisolone 5 mg, Inflagic 20 mg, Kidpred 5 mg/5 ml solution, P-Cort 5 mg & solution, Pedipred solution, Pednil 5/10 mg & solution, Pednisol 5/10/20 mg, Peron 5/10/20 mg, Precodil 2/5/10/20 mg & 5 mg/5 ml or 15 mg/5 ml solution, Pred 5/10/20 mg, Predimax 5/10/20 mg, Prednisolone 5 mg, Rayox 20 mg, Rednisone 5 mg, Solone 5/10/20 mg & solution, Sonipred 5/10/20 mg & solution, Zenilon 5 mg।

8.2 Methylprednisolone sodium succinate — IV pulse

  1. Generic name: Methylprednisolone sodium succinate
  2. Therapeutic category: Systemic corticosteroid
  3. Pharmacological class: Potent glucocorticoid
  4. Why used: Severe myositis, dysphagia, myocarditis, rapidly progressive ILD, severe flare—rapid control।
  5. Dose: 500 mg–1 g IV daily for 3 days, তারপর oral prednisolone regimen। Rapidly progressive ILD-তে ACR guideline pulse IV methylprednisolone first-line option হিসেবে recommend করে।
  6. Duration: 3 days pulse; repeat specialist decision।
  7. Side effects: Hyperglycemia, hypertension, psychosis, infection, electrolyte imbalance, GI bleed।
  8. Interaction: NSAID/anticoagulant GI bleed risk; vaccines; CYP3A interacting drugs।
  9. Bangladesh brand — Medex listed: Mepcort 500 mg/1 g vial, Methipred 500 mg/1 g vial, Methsolon 500 mg/1 g vial, Pro-Medrol 500 mg/1 g vial, Solu-Medrol 40/125/500 mg/1 g vial, Solupred 125/500 mg/1 g vial।

8.3 Methotrexate

  1. Generic name: Methotrexate
  2. Therapeutic category: csDMARD / immunosuppressant
  3. Pharmacological class: Antimetabolite; folate antagonist; DHFR inhibitor। Medex pharmacology অনুযায়ী methotrexate dihydrofolate reductase inhibit করে DNA synthesis/repair/cellular replication কমায়।
  4. Why used: Steroid-sparing first-line drug; muscle inflammation ও dermatomyositis skin disease-এ useful।
  5. Dose: 10–15 mg once weekly PO/SC, 2–4 সপ্তাহ পর 20–25 mg once weekly পর্যন্ত increase; কখনো daily নয়।
  6. Duration: Response 6–12 weeks; remission maintain করতে 1–2 years বা longer।
  7. Side effects: Nausea, stomatitis, hepatotoxicity, marrow suppression, pneumonitis, teratogenicity, alopecia।
  8. Interaction: Trimethoprim/co-trimoxazole full dose, alcohol, NSAID high dose, penicillin, PPIs—MTX toxicity risk; pregnancy contraindicated।
  9. Bangladesh brand — Medex listed: MTX injection 50 mg/2 ml, Trexall tablet 2.5/10 mg & injection 50 mg/2 ml/1000 mg vial, Trexonate tablet 2.5/10 mg & injection 50 mg/2 ml/1000 mg vial।

8.4 Folic acid / Folinic acid — MTX toxicity reduction

  1. Generic name: Folic acid; severe MTX toxicity হলে Folinic acid
  2. Therapeutic category: Vitamin / antidote support
  3. Pharmacological class: Folate replacement; folinic acid = reduced folate rescue
  4. Why used: Methotrexate-induced mouth ulcer, GI upset, cytopenia, liver enzyme rise কমাতে।
  5. Dose: Folic acid 5 mg once weekly, usually MTX-এর 24–48 h পরে; অথবা 1 mg daily except MTX day।
  6. Duration: যতদিন MTX চলবে।
  7. Side effects: Generally well tolerated; rare GI upset, hypersensitivity। Medex folic acid well tolerated বলে উল্লেখ করেছে।
  8. Interaction: Significant interaction uncommon; anticonvulsant level affect করতে পারে।
  9. Bangladesh brand — Medex listed: Folic acid: B-9 2.5 mg/5 ml solution, Folac 5 mg, Folate 5 mg, Folcid 5 mg, Foli Kidz solution, Folic Acid 5 mg, Folic OS solution, Folic-Son 5 mg, Folicare solution, Folison 5 mg, Folus 5 mg, Frutifol solution, Refolic 5 mg। Folinic acid: Biofol 5/15 mg, Folcasin injection 10 mg/ml, Folicid injection 10 mg/ml, Folimax injection, Folinex 5/15 mg & injection, Folinic 5 mg & injection, Folipro 5 mg, Folistar 15 mg।

8.5 Azathioprine

  1. Generic name: Azathioprine
  2. Therapeutic category: csDMARD / immunosuppressant
  3. Pharmacological class: Purine antimetabolite
  4. Why used: Steroid-sparing drug; polymyositis/dermatomyositis, overlap disease, ILD maintenance। Medex indication list-এ dermatomyositis ও polymyositis আছে।
  5. Dose: Start 50 mg/day or 1 mg/kg/day, increase to 2–3 mg/kg/day as tolerated। TPMT/NUDT15 if available।
  6. Duration: Response 8–12 weeks; maintenance 1–3 years depending relapse।
  7. Side effects: Nausea, pancreatitis, hepatotoxicity, leukopenia, infection, malignancy risk long term।
  8. Interaction: Allopurinol/febuxostat → severe marrow toxicity; warfarin effect reduce; ACEI/co-trimoxazole marrow risk; live vaccine avoid। Medex also allopurinol, anticoagulants, myelosuppressive agents, live vaccines, ribavirin, methotrexate interactions lists করেছে।
  9. Bangladesh brand — Medex listed: Imruza 25 mg, Imruza 50 mg।

8.6 Mycophenolate mofetil

  1. Generic name: Mycophenolate mofetil
  2. Therapeutic category: Immunosuppressant / steroid-sparing agent
  3. Pharmacological class: IMPDH inhibitor; lymphocyte proliferation inhibitor। Medex description অনুযায়ী MMF IMPDH inhibit করে guanosine nucleotide synthesis কমায় এবং lymphocyte cytostatic effect দেয়।
  4. Why used: Myositis-associated ILD, dermatomyositis skin disease, refractory muscle disease। ACR SARD-ILD guideline first-line ILD options হিসেবে mycophenolate, azathioprine, rituximab, cyclophosphamide recommend করে।
  5. Dose: 500 mg BD, increase every 1–2 weeks to 1–1.5 g BD।
  6. Duration: ILD/skin disease-এ often long-term 1–3 years।
  7. Side effects: Diarrhea, nausea, leukopenia, infection, hepatotoxicity, teratogenicity।
  8. Interaction: Antacid/cholestyramine absorption কমায়; acyclovir/ganciclovir toxicity risk; live vaccine avoid; pregnancy contraindicated।
  9. Bangladesh brand — Medex listed: Cellcept 500 mg, Graftcept 500 mg, MMF 500 mg, Mycophenolate-Mofetil 500 mg, Phenocept 500 mg।

8.7 Hydroxychloroquine sulphate

  1. Generic name: Hydroxychloroquine sulphate
  2. Therapeutic category: DMARD / antimalarial immunomodulator
  3. Pharmacological class: 4-aminoquinoline। Medex pharmacology অনুযায়ী এটি immunosuppressant effect করে acute phase/reactants কমায়।
  4. Why used: Dermatomyositis skin rash, photosensitive rash, arthralgia; isolated muscle weakness control-এর জন্য একা যথেষ্ট নয়।
  5. Dose: 200–400 mg/day PO with food, max ≤5 mg/kg/day actual body weight।
  6. Duration: 3–6 months trial; long-term if skin benefit।
  7. Side effects: Retinopathy, GI upset, skin pigmentation, QT prolongation rare, myopathy rare। Baseline/annual eye screening।
  8. Interaction: QT-prolonging drugs, digoxin level ↑, antidiabetic effect ↑।
  9. Bangladesh brand — Medex listed: Avloquin-H 200 mg, Bioquin 200 mg, Corofree 200 mg, Coroquin 200 mg, Covikil 200 mg, H-Quin 200 mg, HCQ 200 mg, Hydroquin 200 mg, Kovicin 200 mg, Oxiquin 200/400 mg, Quinivir 200 mg, Reconil 200 mg।

8.8 Tacrolimus

  1. Generic name: Tacrolimus monohydrate oral
  2. Therapeutic category: Immunosuppressant
  3. Pharmacological class: Calcineurin inhibitor
  4. Why used: Antisynthetase syndrome / IIM-ILD, refractory ILD, anti-MDA5 rapidly progressive ILD context। ACR guideline IIM-ILD-এ calcineurin inhibitor first-line/progression option হিসেবে conditionally recommend করে।
  5. Dose: 1 mg BD, titrate; common target trough 5–10 ng/ml; renal function/BP/K+ monitor।
  6. Duration: ILD response অনুযায়ী months–years।
  7. Side effects: Nephrotoxicity, hypertension, hyperkalemia, tremor, diabetes, infection।
  8. Interaction: CYP3A inhibitors—clarithromycin, azole antifungal, diltiazem, grapefruit ↑ level; rifampicin/phenytoin ↓ level; ACEI/ARB/spironolactone hyperkalemia risk।
  9. Bangladesh brand — Medex listed: Crilomus 0.5/1 mg, Tacrocap 0.5/1 mg, Tacrograf 0.5 mg; Medex filter shows 0.5 mg and 1 mg strengths.

8.9 Cyclosporine oral

  1. Generic name: Cyclosporine oral
  2. Therapeutic category: Immunosuppressant
  3. Pharmacological class: Calcineurin inhibitor। Medex describes cyclosporine as potent immunosuppressive cyclic polypeptide acting reversibly on lymphocytes।
  4. Why used: IIM-ILD/antisynthetase ILD alternative to tacrolimus।
  5. Dose: 2.5–5 mg/kg/day PO divided BD, trough level/renal function/BP guided।
  6. Duration: Specialist-directed months–years।
  7. Side effects: Nephrotoxicity, hypertension, hypertrichosis, gingival hyperplasia, tremor, hyperkalemia।
  8. Interaction: Same CYP3A interactions; NSAID/aminoglycoside nephrotoxicity ↑; statin myopathy risk ↑।
  9. Bangladesh brand — Medex listed: Neoral 25/50/100 mg capsule, Sporium syrup 100 mg/ml।

8.10 Cyclophosphamide

  1. Generic name: Cyclophosphamide
  2. Therapeutic category: Cytotoxic immunosuppressant
  3. Pharmacological class: Alkylating agent; Medex pharmacology says active metabolites DNA cross-linking করে।
  4. Why used: Severe/refractory myositis, rapidly progressive ILD, life-threatening organ disease। BSR severe/refractory IIM-এ cyclophosphamide consider করতে বলে, এবং IV pulse oral-এর তুলনায় fewer side effects দিতে পারে।
  5. Dose: IV pulse 500–1000 mg/m² monthly for 6 months or 10–15 mg/kg every 2–4 weeks; hydration ± mesna depending dose/protocol।
  6. Duration: Usually 3–6 pulses or 6 months, then safer maintenance DMARD।
  7. Side effects: Leukopenia, infection, hemorrhagic cystitis, infertility, alopecia, nausea, malignancy risk।
  8. Interaction: Live vaccine avoid; myelosuppressive drugs additive; warfarin variable; CYP drugs affect activation।
  9. Bangladesh brand — Medex listed: Cycloph 200/500 mg/1 g vial, Cyclotox 200 mg/1 g vial, Cyphos 200 mg/1 g vial, Endoxan 50 mg tablet।

8.11 Rituximab

  1. Generic name: Rituximab
  2. Therapeutic category: Biologic immunosuppressant
  3. Pharmacological class: Anti-CD20 monoclonal antibody। Medex describes rituximab as chimeric monoclonal IgG1 antibody directed against CD20 antigen।
  4. Why used: Refractory myositis, autoantibody-positive disease, refractory skin disease, ILD। BSR recommends considering rituximab in refractory myositis and skin disease refractory to glucocorticoid/csDMARD।
  5. Dose: Common rheumatology regimen 1 g IV day 1 and day 15, repeat after 6 months based on disease; alternative 375 mg/m² weekly × 4।
  6. Duration: Repeat cycle every 6–12 months if relapse/active disease।
  7. Side effects: Infusion reaction, HBV reactivation, hypogammaglobulinemia, infection, rare PML।
  8. Interaction: Live vaccine avoid; other immunosuppressants infection risk ↑। Screen HBV/HCV/TB where appropriate। Medex notes infusion should be by healthcare professional with support for severe infusion reactions।
  9. Bangladesh brand — Medex listed: Mabthera 10 mg/ml 100/500 mg vial, Recelbia 10 mg/ml 100/500 mg vial, Relyto 10 mg/ml 100/500 mg vial, Ritumab 10 mg/ml 100/500 mg vial, Rituris 10 mg/ml 100/500 mg vial, Rituxim 10 mg/ml 100/500 mg vial, Truxima 10 mg/ml 100/500 mg vial।

8.12 IVIG — Human immunoglobulin-G

  1. Generic name: Human immunoglobulin-G / IVIG
  2. Therapeutic category: Immunomodulator
  3. Pharmacological class: Pooled human IgG preparation; Medex describes it as purified IgG from human plasma pools।
  4. Why used: Severe/refractory dermatomyositis, dysphagia, rapidly progressive weakness, refractory skin disease; BSR dysphagia-resistant active disease-এ IVIG consider করতে বলে।
  5. Dose: 2 g/kg per course, divided over 2–5 days, repeat every 4 weeks for 3–6 months then reassess।
  6. Duration: 3–6 cycles commonly; long-term maintenance selected cases।
  7. Side effects: Headache, fever, aseptic meningitis, thrombosis, renal dysfunction, hemolysis, infusion reaction।
  8. Interaction: Live vaccines—MMR/varicella response reduce for months; nephrotoxic drugs caution।
  9. Bangladesh brand — Medex listed: Humaglobin 2.5 g/50 ml, Normoglobin 500 mg/10 ml and 2.5 g/50 ml, Pentaglobin 500 mg/10 ml, 2.5 g/50 ml, 5 g/100 ml।

8.13 Sulphamethoxazole + Trimethoprim — PJP prophylaxis

  1. Generic name: Sulphamethoxazole + Trimethoprim
  2. Therapeutic category: Antimicrobial prophylaxis
  3. Pharmacological class: Sulfonamide + dihydrofolate reductase inhibitor; Medex therapeutic class: sulphonamides & trimethoprim।
  4. Why used: Prednisolone ≥20 mg/day for ≥4 weeks + another immunosuppressant/rituximab/cyclophosphamide/ILD হলে Pneumocystis jirovecii pneumonia prevention।
  5. Dose: DS 800/160 mg PO 3 times weekly or daily, depending risk/tolerance।
  6. Duration: High-dose steroid/immunosuppression risk কমা পর্যন্ত।
  7. Side effects: Rash, SJS/TEN rare, cytopenia, hyperkalemia, renal dysfunction, GI upset।
  8. Interaction: Warfarin ↑ INR, ACEI/ARB/spironolactone hyperkalemia, methotrexate marrow toxicity risk—MTX রোগীতে caution। Medex warns prolonged full dose can cause macrocytic anemia and sulfonylurea potentiation।
  9. Bangladesh brand — Medex listed: Alcot DS 800/160 mg, Apcetrim 400/80 mg & suspension, Biotrim 400/80 mg & suspension, Biotrim DS 800/160 mg, Cotrimox 400/80 mg & suspension, Cotrimox DS 800/160 mg, Cots suspension; Medex lists many companies/brands।

8.14 Calcium + Vitamin D3 ± Alendronate — steroid bone protection

  1. Generic name: Calcium + Vitamin D3; Alendronic acid ± Vitamin D3
  2. Therapeutic category: Bone protection / anti-osteoporotic
  3. Pharmacological class: Mineral-vitamin supplement; bisphosphonate। Alendronate osteoclast-mediated bone resorption inhibit করে।
  4. Why used: Long-term steroid-induced osteoporosis/fracture prevention।
  5. Dose: Calcium elemental 1000–1200 mg/day + Vitamin D3 800–1000 IU/day; high-risk হলে Alendronate 70 mg once weekly বা guideline-based alternative। Medex alendronate osteoporosis dose 70 mg once weekly উল্লেখ করেছে।
  6. Duration: Steroid চলাকালীন; DEXA/FRAX অনুযায়ী।
  7. Side effects: Calcium—constipation, hypercalcemia; alendronate—esophagitis, dyspepsia, musculoskeletal pain, rare ONJ/atypical femur fracture।
  8. Interaction: Calcium iron/levothyroxine/quinolone/tetracycline absorption কমায়; alendronate calcium/antacid/food-এর সাথে absorption কমে, NSAID GI side effect বাড়ে।
  9. Bangladesh brand — Medex listed examples: Calcium + D3: Calci-D Plus 500 mg+200 IU, Calcibo D 500 mg+200 IU, Calcium D 500 mg+200 IU, Casalt-D 500 mg+200 IU, Ostoplus-D 500 mg+200 IU, Ostoplus-DX 600 mg+400 IU, Protebon-D 500 mg+200 IU। Alendronic acid: Alendon 10/70 mg; Alendronic acid + D3: Bonemass D 70 mg+2800 IU, Ostel-D 70 mg+2800 IU।

8.15 Pantoprazole — GI protection when indicated

  1. Generic name: Pantoprazole sodium
  2. Therapeutic category: Anti-ulcer / gastroprotection
  3. Pharmacological class: Proton pump inhibitor
  4. Why used: High-dose steroid + NSAID/antiplatelet/ulcer risk থাকলে gastric protection; routine all patients নয়।
  5. Dose: 20–40 mg OD, breakfast-এর আগে; severe inpatient GI risk হলে IV 40 mg OD/BD।
  6. Duration: GI risk যতদিন থাকে; unnecessary long-term avoid।
  7. Side effects: Headache, diarrhea, hypomagnesemia, B12/iron malabsorption long term, C. difficile risk।
  8. Interaction: Clopidogrel interaction omeprazole-এর তুলনায় কম; high-dose MTX toxicity concern with PPIs—monitor।
  9. Bangladesh brand — Medex listed examples: Pantid 20/40 mg & IV 40 mg, Pantobex 20/40 mg & IV 40 mg, Pantodac 20/40 mg & IV 40 mg, Pantogen 20/40 mg, Pantogut 20/40 mg & IV 40 mg, Pantolok 20/40 mg & IV 40 mg, Pantonix 20/40 mg & IV 40 mg।

9) Non-pharmacological management

  • Physiotherapy / graded exercise
    • Acute severe weaknessে supervised gentle ROM।
    • Disease control হলে progressive resistance + aerobic exercise।
    • BSR guideline specialist physiotherapist/occupational therapist-led exercise program recommend করে; exercise safe and effective, function ও quality of life improve করতে পারে।
  • Sun protection
    • Dermatomyositis flare কমাতে broad-spectrum SPF ≥50 sunscreen, hat, full sleeve, midday sun avoid।
    • BSR sun avoidance and high-factor broad-spectrum sunscreen consider করতে বলে।
  • Swallow safety
    • Dysphagia থাকলে speech/swallow therapist, thickened fluid, aspiration precaution।
    • Severe aspiration হলে admission।
  • ILD care
    • HRCT/PFT-DLCO baseline and follow-up।
    • Smoking cessation, influenza/pneumococcal/COVID vaccine before heavy immunosuppression if possible।
  • Cancer screening
    • Adult dermatomyositis/IIM-এ age-appropriate plus risk-based enhanced screening।

10) Prevention — কীভাবে করব

  • Autoimmune myositis পুরোপুরি prevent করা যায় না, তবে severe complication prevent করা যায়।
  • Early detection: proximal weakness + CK rise + rash হলে delay না করে evaluation।
  • Statin/colchicine/alcohol/drug-induced myopathy suspicion হলে offending drug review।
  • Sun avoidance in dermatomyositis।
  • Vaccination before rituximab/cyclophosphamide/high-dose steroid where feasible; live vaccine avoid during major immunosuppression।
  • Osteoporosis prevention: calcium-vitamin D, DEXA, bisphosphonate if indicated।
  • Infection prevention: PJP prophylaxis in high-risk immunosuppression, TB/HBV/HCV screen before biologic/cyclophosphamide/rituximab।
  • Regular monitoring: CK, CBC, LFT, creatinine, urine, BP, glucose, PFT/HRCT, eye check for HCQ।

11) Overview / Summary

  • Myositis হলো muscle inflammation; clinical hallmark symmetrical proximal muscle weakness।
  • Most important causes are autoimmune IIM: dermatomyositis, polymyositis, necrotizing myopathy, antisynthetase syndrome, inclusion body myositis।
  • Diagnosis needs clinical pattern + CK/aldolase + autoantibody + EMG/MRI + biopsy when needed + ILD/cardiac/cancer screening।
  • Treatment core: high-dose steroid induction + early steroid-sparing DMARD; severe/refractory disease হলে IV methylpred, MMF/tacrolimus/cyclophosphamide/rituximab/IVIG।
  • Monitoring and prevention are essential because mortality/morbidity mainly ILD, dysphagia/aspiration, infection, malignancy, cardiac involvement থেকে আসে।

12) Actionable Plan / Steps

  1. Suspected case: CK, aldolase, AST/ALT, LDH, CBC, ESR/CRP, RFT/LFT, urine, TSH, vitamin D।
  2. Subtype confirmation: ANA/ENA + myositis antibody panel।
  3. Organ screen: HRCT chest + PFT/DLCO; ECG/troponin-I/echo; dysphagia assessment।
  4. Imaging/biopsy: MRI thigh/shoulder; biopsy if diagnosis uncertain or subtype needed।
  5. Start treatment if active disease: Prednisolone 0.5–1 mg/kg/day ± IV methylpred pulse if severe।
  6. Add steroid-sparing early: MTX/AZA; ILD হলে MMF/tacrolimus/cyclosporine preferred by phenotype।
  7. Refractory/severe: Cyclophosphamide, rituximab, IVIG specialist centre decision।
  8. Prevent complications: PJP prophylaxis when high risk, Ca-D ± bisphosphonate, vaccination, physiotherapy, sun protection, cancer screening।
  9. Monitor: CK + strength every 4–8 weeks initially; CBC/LFT/RFT drug-specific; PFT/HRCT as ILD course demands।

13) Reference List — Textbook + Guideline + DOI

Textbooks

  • Harrison’s Principles of Internal Medicine, 21st ed. Chapter: Inflammatory Myopathies / Disorders of Muscle.
  • Davidson’s Principles and Practice of Medicine, 24th ed. Chapter: Rheumatology / Neuromuscular disorders.
  • Robbins & Cotran Pathologic Basis of Disease, 10th ed. Chapter: Skeletal Muscle; inflammatory myopathies pathology.
  • Katzung & Trevor’s Basic & Clinical Pharmacology, 16th ed. Chapters: Adrenocorticosteroids, immunosuppressants, antimetabolites, biologics.
  • Goodman & Gilman’s The Pharmacological Basis of Therapeutics, 14th ed. Chapters: Immunosuppressants, antirheumatic drugs, glucocorticoids.

Guidelines

  • British Society for Rheumatology guideline on management of paediatric, adolescent and adult patients with idiopathic inflammatory myopathy, 2022. DOI: 10.1093/rheumatology/keac115.
  • 2017 EULAR/ACR Classification Criteria for Adult and Juvenile Idiopathic Inflammatory Myopathies. DOI: 10.1136/annrheumdis-2017-211468 / methodology DOI: 10.1136/rmdopen-2017-000507.
  • 2023 ACR/CHEST guideline for SARD-associated ILD treatment. DOI: 10.1002/art.42861.
  • 2023 ACR/CHEST guideline for SARD-ILD screening and monitoring. DOI: 10.1002/art.42860.

Peer-reviewed DOI

  • Aggarwal R, et al. Trial of Intravenous Immune Globulin in Dermatomyositis. N Engl J Med. 2022. DOI: 10.1056/NEJMoa2117912.
  • Oddis CV, et al. Rituximab in refractory adult and juvenile dermatomyositis and adult polymyositis. Arthritis Rheum. 2013. DOI: 10.1002/art.37754.

Md.Rafiqul Islam Lipu,Date:-15-06-2026 Wednesday,Time:-09-49-12 Am

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