A 35-year-old woman from rural India presents with recurrent oral and genital ulcers, ocular inflammation (uveitis), and erythema nodosum. Pathergy test is positive. What is the drug of choice for initial management of systemic manifestations?

Question

Accepted Answer

C. Prednisolone. ## Behçet's Disease — First-Line Systemic Therapy

### Clinical Presentation
This patient has classic Behçet's disease (BD): recurrent oral and genital ulcers, ocular involvement (uveitis), systemic inflammation (erythema nodosum), and positive pathergy test. BD is more common in patients from Mediterranean, Middle Eastern, and Asian regions (including India).

### Drug of Choice: Corticosteroids (Prednisolone)

**Key Point:** Systemic corticosteroids are first-line for acute flares and systemic manifestations of Behçet's disease, especially those affecting eyes, CNS, or vasculature.

**High-Yield:** Treatment algorithm for BD:

```mermaid
flowchart TD
  A[Behçet's Disease Diagnosed]:::outcome --> B{Severity & Organ Involvement?}:::decision
  B -->|Mucocutaneous only| C[Topical corticosteroids + Colchicine]:::action
  B -->|Ocular/Systemic/Severe| D[Systemic Prednisolone 0.5-1 mg/kg/day]:::action
  D --> E[Response in 2-4 weeks?]:::decision
  E -->|Yes| F[Taper prednisolone over 3-6 months]:::action
  E -->|No or Steroid-dependent| G[Add steroid-sparer: Azathioprine or Cyclosporine]:::action
  C --> H[Maintenance: Colchicine 1-1.5 mg/day]:::action
```

### Role of Each Agent

| Agent | Indication | Dose | Notes |
|-------|-----------|------|-------|
| **Prednisolone** | Systemic/ocular flares | 0.5–1 mg/kg/day | First-line for acute inflammation |
| **Colchicine** | Mucocutaneous disease, maintenance | 1–1.5 mg/day | Prophylaxis for recurrent ulcers; minimal systemic effects |
| **Azathioprine** | Steroid-sparing, ocular/systemic | 1–2.5 mg/kg/day | Added if steroid-dependent or for long-term control |
| **Cyclosporine** | Severe ocular/CNS disease | 3–5 mg/kg/day | Reserved for vision-threatening uveitis or CNS involvement |

**Clinical Pearl:** In this case, the patient has systemic manifestations (erythema nodosum, uveitis). Prednisolone is essential for rapid anti-inflammatory effect. Colchicine alone is insufficient for systemic disease.

**Warning:** Colchicine monotherapy is appropriate ONLY for recurrent mucocutaneous ulcers without systemic involvement. This patient requires systemic corticosteroids.

**Mnemonic — BD Treatment Tiers:** **CCAS**
- **C** — Colchicine (mucocutaneous, prophylaxis)
- **C** — Corticosteroids (acute systemic/ocular flares)
- **A** — Azathioprine (steroid-sparer)
- **S** — Severe cases → Cyclosporine or TNF-α inhibitors

Answer

A. Azathioprine

Answer

B. Cyclosporine

Answer

D. Colchicine

A 35-year-old woman from rural India presents with recurrent oral and genital ulcers, ocular inflammation (uveitis), and erythema nodosum. Pathergy test is positive. What is the drug of choice for initial management of systemic manifestations?

Explanation

Behçet's Disease — First-Line Systemic Therapy

Clinical Presentation

Drug of Choice: Corticosteroids (Prednisolone)

Role of Each Agent

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Agent	Indication	Dose	Notes
Prednisolone	Systemic/ocular flares	0.5–1 mg/kg/day	First-line for acute inflammation
Colchicine	Mucocutaneous disease, maintenance	1–1.5 mg/day	Prophylaxis for recurrent ulcers; minimal systemic effects
Azathioprine	Steroid-sparing, ocular/systemic	1–2.5 mg/kg/day	Added if steroid-dependent or for long-term control
Cyclosporine	Severe ocular/CNS disease	3–5 mg/kg/day	Reserved for vision-threatening uveitis or CNS involvement