Rheumatology and Immunology Practice Questions

Q: A 26 year old female patient presented with fever, oral ulcers, sensitivity to light and rash over the malar area of the face sparing the nasolabial folds of both side. Which of the following indicates the condition associated with these manifestations?

SLE. ***SLE*** - The combination of **fever**, **oral ulcers**, **photosensitivity**, and a **malar rash** (which typically spares the nasolabial folds), particularly in a young female, is classic for **Systemic Lupus Erythematosus (SLE)** [1]. - SLE is a **chronic autoimmune inflammatory disease** that can affect multiple organ systems [2]. *Rosacea* - Rosacea often presents with **facial erythema**, **telangiectasias**, and papulopustules, primarily on the central face, but it does not typically involve oral ulcers, fever, or photosensitivity in the same way as SLE [4]. - The rash of rosacea is usually not a classic malar rash sparing the nasolabial folds, and it is not an autoimmune systemic disease. *Dermatomyositis* - Dermatomyositis is characterized by **proximal muscle weakness** and specific skin manifestations like **Gottron's papules** (over joints), **heliotrope rash** (periorbital edema), and a **shawl sign**, which differ from the presented symptoms [3]. - While it can cause light sensitivity and a rash, the distribution and associated symptoms (like no mention of muscle weakness) are not typical for a primary presentation of dermatomyositis. *Psoriasis* - Psoriasis typically presents with **well-demarcated erythematous plaques** covered with **silvery scales**, commonly on extensor surfaces like elbows and knees, and can also affect nails and joints. - It does not typically cause fever, oral ulcers, or a malar rash with nasolabial fold sparing, which are hallmarks of SLE.

Q: A patient with rheumatoid arthritis on Methotrexate, steroids and NSAIDs for past 4 months has had no retardation of disease progression. What is the next rational step in management?

Add Sulfasalazine. Focus on **Add Sulfasalazine**: - Since the patient has not responded to **Methotrexate** alone, adding a second conventional synthetic **disease-modifying antirheumatic drug (DMARD)** like **Sulfasalazine** is a common and appropriate step in a **treat-to-target strategy** for rheumatoid arthritis [1]. - This approach aims to achieve **disease remission** or **low disease activity** by combining therapies to enhance efficacy. *Continue Methotrexate and steroids at higher dose* - Increasing the dose of **Methotrexate** might be considered if the current dose is sub-optimal, but after 4 months with no improvement, simply continuing current medications at higher doses without adding another agent is less likely to significantly alter **disease progression** [1]. - Prolonged higher-dose **steroids** carry significant risks and are generally used for symptom control, not primary disease modification. *Stop oral Methotrexate and start parenteral Methotrexate* - Switching to **parenteral Methotrexate** is considered if **oral Methotrexate** absorption is an issue or if the patient experiences gastrointestinal side effects [1]. - While parenteral administration can improve bioavailability, it doesn't represent a change in therapeutic strategy for **uncontrolled disease activity**. *Start treatment with anti-TNF alpha drugs* - **Biologic DMARDs** like **anti-TNF alpha drugs** are typically reserved for patients who have failed **two or more conventional synthetic DMARDs**, including **Methotrexate**, or in cases of severe, rapidly progressing disease [2]. - While effective, they are more expensive and have different side effect profiles, making them a later-line treatment in the management algorithm [2].

Q: Which of the following is true about polyaeritis nodosa?

HBsAg is positive in 30% patients. ### HBsAg is positive in 30% patients - **Polyarteritis nodosa (PAN)** is strongly associated with **hepatitis B virus (HBV)** infection; about 30% of patients with PAN have evidence of current or past HBV infection, particularly **HBsAg positivity**. - This association suggests that HBV infection can trigger the immune complex vasculitis characteristic of PAN. ### It shows fibrinoid necrosis in large blood vessels - PAN primarily affects **medium-sized muscular arteries**, not typically large blood vessels [1]. - The inflammation causes **fibrinoid necrosis** and aneurysmal dilations in these medium-sized arteries [1]. ### It has ANCA positivity - **Polyarteritis nodosa (PAN)** is generally considered an **ANCA-negative vasculitis**. - **ANCA positivity** (especially c-ANCA/PR3-ANCA or p-ANCA/MPO-ANCA) is characteristic of other small-vessel vasculitides like **Granulomatosis with polyangiitis** or **Microscopic polyangiitis**. ### Affected individuals have involvement of pulmonary circulation. - A defining characteristic of **Polyarteritis nodosa (PAN)** is that it generally **spares the pulmonary circulation** [1]. - Pulmonary involvement is more commonly seen in other vasculitides, such as **Granulomatosis with polyangiitis (Wegener's)** or **Eosinophilic granulomatosis with polyangiitis (Churg-Strauss)**.

Q: A 35-year-old woman with history of melanoma develops new-onset facial telangiectasias and sclerodactyly. ANA is positive. Most appropriate next step for staging is:

High-resolution chest CT. ***High-resolution chest CT*** - The patient's symptoms (telangiectasias, sclerodactyly, positive ANA) are highly suggestive of **systemic sclerosis (scleroderma)**, a connective tissue disease with significant pulmonary involvement [1]. - **Interstitial lung disease (ILD)**, a common and serious complication of systemic sclerosis, requires **high-resolution CT (HRCT)** for proper staging and monitoring. *Skin biopsy only* - While a skin biopsy might confirm cutaneous involvement of systemic sclerosis, it does not assess the crucial systemic complications, particularly pulmonary involvement. - It would therefore be insufficient for comprehensive staging in a patient with suspected scleroderma. *Echocardiogram with PFTs* - An **echocardiogram** would assess for **pulmonary hypertension** and other cardiac involvement, which are also complications of scleroderma. - **Pulmonary function tests (PFTs)** are important for evaluating the severity of ILD, but they do not provide the detailed anatomical information needed for initial staging that HRCT offers. *PET scan* - A **PET scan** is primarily used in oncology for staging and surveillance of malignancies, particularly given the patient's history of melanoma. - While melanoma could metastasize, the **new-onset telangiectasias and sclerodactyly**, along with a **positive ANA**, point much more strongly to a new rheumatologic condition like systemic sclerosis, for which PET is not the primary staging modality.

Q: A 28-year-old woman presents with new-onset blisters on sun-exposed areas and malar rash. Labs show ANA 1:320, anti-dsDNA positive, low C3/C4. Skin biopsy shows subepidermal blister with neutrophilic infiltrate. Most appropriate initial treatment is:

Hydroxychloroquine plus systemic steroids. ***Hydroxychloroquine plus systemic steroids*** - The patient presents with **lupus flares** characterized by a malar rash and positive **anti-dsDNA antibodies** in the presence of immune complex-mediated skin disease (blisters on sun-exposed areas) and hypocomplementemia (low C3/C4) [1]. - **Hydroxychloroquine** is a standard treatment for **cutaneous lupus** and helps prevent systemic flares, while **systemic steroids** are appropriate for managing acute, severe inflammation and blisters [1]. *Dapsone alone* - While **dapsone** can be used for blistering skin conditions, particularly **dermatitis herpetiformis** or some lupus-associated bullous lesions, it is insufficient as monotherapy given the systemic nature of her lupus with active disease (malar rash, positive anti-dsDNA, hypocomplementemia). - It does not address the underlying systemic immunological dysregulation or the extent of the skin involvement in this patient. *Hydroxychloroquine alone* - **Hydroxychloroquine** is a cornerstone of lupus treatment, particularly for cutaneous symptoms and preventing flares [1]. However, for acute, severe manifestations like extensive blistering lesions and severe malar rash, especially with systemic involvement (positive anti-dsDNA, low complement), it is often not sufficient on its own to achieve rapid control of inflammation. - Adding **systemic steroids** is crucial for prompt resolution of acute, severe symptoms and to prevent organ damage [1]. *Mycophenolate mofetil* - **Mycophenolate mofetil (MMF)** is an immunosuppressant typically reserved for more severe manifestations of lupus, such as **lupus nephritis** or other organ-threatening disease, or as a steroid-sparing agent. - While it could be considered in refractory cases, it is not the **initial treatment** of choice for an acute, severe flare dominated by cutaneous symptoms, where oral steroids provide more rapid relief.

Q: A patient with rheumatoid arthritis presents with sudden onset wrist drop. What is the most likely cause?

Tendon Rupture. ***Tendon Rupture*** - In rheumatoid arthritis, **chronic inflammation** can lead to weakening and eventual rupture of tendons, particularly the extensor tendons of the wrist, resulting in a **wrist drop**. [1] - The **sudden onset** of wrist drop is highly suggestive of a mechanical failure like a tendon rupture, as opposed to a more gradual process. *Joint Destruction* - While rheumatoid arthritis causes significant joint destruction, it typically manifests as pain, deformity, and reduced range of motion, not usually a **sudden-onset functional deficit** like wrist drop. - Joint destruction can contribute to tendon dysfunction but is not the direct, most likely cause of a **sudden wrist drop**. *Muscle Weakness* - Muscle weakness in rheumatoid arthritis often results from **disuse atrophy**, **steroid myopathy**, or **direct inflammatory effects**, but it tends to be more generalized or gradual. [1] - It would not typically cause a **sudden, focally disabling symptom** like a wrist drop in a specific part of the limb. *Nerve Compression* - **Nerve compression**, such as **carpal tunnel syndrome** (median nerve) or **ulnar nerve entrapment**, can occur in RA. - However, nerve compression leading to a wrist drop would typically involve the **radial nerve**, and while possible, **tendon rupture** is a more common and classic cause of sudden wrist drop in RA due to chronic inflammation.

Q: A 25-year-old presents with back pain, morning stiffness >1 hour, and restricted chest expansion. What is the most likely HLA association?

HLA-B27. ***HLA-B27*** - The presentation of **back pain**, morning stiffness lasting **over 1 hour**, and **restricted chest expansion** are classic symptoms of **ankylosing spondylitis**. [1] - **Ankylosing spondylitis** has a very strong association with the presence of the **HLA-B27** allele. [1] *HLA-DQ2* - **HLA-DQ2** (along with HLA-DQ8) is strongly associated with **celiac disease**, an autoimmune disorder affecting the small intestine. - Its association is not with inflammatory back pain or restricted chest expansion as seen in this patient. *HLA-B51* - **HLA-B51** is the most significant genetic risk factor for **Behçet's disease**, a chronic inflammatory disorder that causes oral and genital ulcers, as well as eye and skin lesions. - It is not typically linked to spondyloarthropathies like ankylosing spondylitis. [1] *HLA-DR4* - **HLA-DR4** is a major genetic susceptibility factor for **rheumatoid arthritis**, an autoimmune disease primarily affecting the small joints of the hands and feet. - While rheumatoid arthritis can cause morning stiffness, it rarely presents with restricted chest expansion due to spinal involvement.

Q: Regarding Sjögren's syndrome, all are true except:

Epiphora. ***Epiphora*** - **Epiphora** refers to excessive tearing or watery eyes, which is not characteristic of Sjögren's syndrome. - Sjögren's syndrome is instead characterized by **dry eyes** due to decreased tear production. *Rheumatoid arthritis* - Sjögren's syndrome can occur as a **secondary condition** associated with other autoimmune diseases, including rheumatoid arthritis [1]. - This association means that individuals with rheumatoid arthritis have an increased risk of developing Sjögren's syndrome [1]. *Keratoconjunctivitis sicca* - **Keratoconjunctivitis sicca** (dry eyes) is a hallmark symptom of Sjögren's syndrome, resulting from inflammation and damage to the lacrimal glands [1]. - Patients experience chronic dryness, foreign body sensation, and irritation due to insufficient tear production [1]. *Autoimmune in nature* - Sjögren's syndrome is an **autoimmune disease** where the immune system mistakenly attacks the body's own moisture-producing glands, primarily the lacrimal and salivary glands. - This autoimmune attack leads to chronic inflammation and dysfunction of these glands, causing symptoms of dryness.

Q: Which of the following cannot be diagnosed without positive ANA?

SLE. ***SLE*** - A positive **Antinuclear Antibody (ANA)** test is a mandatory classification criterion for **Systemic Lupus Erythematosus (SLE)**; without it, the diagnosis cannot be made [1]. - While other criteria exist, a **negative ANA** essentially rules out SLE in almost all cases due to its high sensitivity [2]. *Sjogren syndrome* - Although **ANA** is often positive in **Sjögren's syndrome** (especially in a speckled pattern, associated with **anti-Ro/SSA** and **anti-La/SSB**), it is not an absolute requirement for diagnosis [1]. - Diagnosis can be made based on clinical features (dry eyes, dry mouth), positive minor salivary gland biopsy, and other autoantibodies. *Drug induced lupus* - **Drug-induced lupus** also typically presents with a **positive ANA**, frequently with a **homogenous pattern** and often associated with **anti-histone antibodies**. - However, like Sjögren's, a positive ANA is very common but not an absolute prerequisite; clinical context and resolution upon drug cessation are also crucial. *None of the above* - This option is incorrect because **SLE** specifically requires a positive **ANA** for diagnosis according to established classification criteria.

Question 1

A 26 year old female patient presented with fever, oral ulcers, sensitivity to light and rash over the malar area of the face sparing the nasolabial folds of both side. Which of the following indicates the condition associated with these manifestations?

Accepted Answer

SLE

Answer

Rosacea

Answer

Dermatomyositis

Answer

Psoriasis

Question 2

A patient with rheumatoid arthritis on Methotrexate, steroids and NSAIDs for past 4 months has had no retardation of disease progression. What is the next rational step in management?

Accepted Answer

Add Sulfasalazine

Answer

Continue Methotrexate and steroids at higher dose

Answer

Stop oral Methotrexate and start parenteral Methotrexate

Answer

Start treatment with anti-TNF alpha drugs

Question 3

Which of the following is true about polyaeritis nodosa?

Accepted Answer

HBsAg is positive in 30% patients

Answer

It shows fibrinoid necrosis in large blood vessels

Answer

It has ANCA positivity

Answer

Affected individuals have involvement of pulmonary circulation.

Question 4

A 35-year-old woman with history of melanoma develops new-onset facial telangiectasias and sclerodactyly. ANA is positive. Most appropriate next step for staging is:

Accepted Answer

High-resolution chest CT

Answer

Skin biopsy only

Answer

Echocardiogram with PFTs

Answer

PET scan

Question 5

A 62-year-old woman with rheumatoid arthritis presents with painful lower leg ulcers, livedo reticularis, and palpable purpura. Labs show high RF, low C3/C4, positive cryoglobulins. Most appropriate first-line treatment is:

Accepted Answer

Rituximab

Answer

Prednisone alone

Answer

Cyclophosphamide

Answer

Methotrexate

Question 6

A 28-year-old woman presents with new-onset blisters on sun-exposed areas and malar rash. Labs show ANA 1:320, anti-dsDNA positive, low C3/C4. Skin biopsy shows subepidermal blister with neutrophilic infiltrate. Most appropriate initial treatment is:

Accepted Answer

Hydroxychloroquine plus systemic steroids

Answer

Dapsone alone

Answer

Hydroxychloroquine alone

Answer

Mycophenolate mofetil

Question 7

A patient with rheumatoid arthritis presents with sudden onset wrist drop. What is the most likely cause?

Accepted Answer

Tendon Rupture

Answer

Joint Destruction

Answer

Muscle Weakness

Answer

Nerve Compression

Question 8

A 25-year-old presents with back pain, morning stiffness >1 hour, and restricted chest expansion. What is the most likely HLA association?

Accepted Answer

HLA-B27

Answer

HLA-DQ2

Answer

HLA-B51

Answer

HLA-DR4

Question 9

Regarding Sjögren's syndrome, all are true except:

Accepted Answer

Epiphora

Answer

Rheumatoid arthritis

Answer

Keratoconjunctivitis sicca

Answer

Autoimmune in nature

Question 10

Which of the following cannot be diagnosed without positive ANA?

Accepted Answer

SLE

Answer

Sjogren syndrome

Answer

Drug induced lupus

Answer

None of the above

Rheumatology and Immunology — MCQs

Rheumatology and Immunology — MCQs

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