Rheumatology and Immunology Practice Questions

Q: Which of the following conditions is associated with HLA-B27?

Reiter's Syndrome. **Explanation:** **Reiter’s Syndrome (Reactive Arthritis)** is the correct answer because it belongs to the group of **Seronegative Spondyloarthropathies (SpA)**. These conditions are characterized by the absence of Rheumatoid Factor (RF) and a strong genetic association with the **HLA-B27** allele [1]. Reiter’s Syndrome typically presents as a triad of urethritis, conjunctivitis, and arthritis ("Can't see, can't pee, can't climb a tree") following a gastrointestinal or urogenital infection [1][2]. **Analysis of Incorrect Options:** * **A. Osteoarthritis:** This is a degenerative joint disease caused by "wear and tear" of articular cartilage. It is not an immune-mediated inflammatory condition and has no association with HLA-B27. * **B. Sjogren Syndrome:** This is an autoimmune destruction of exocrine glands [1]. It is primarily associated with **HLA-DR3** and **HLA-DQ2**, as well as antibodies like Anti-Ro (SS-A) and Anti-La (SS-B). * **C. Behcet Disease:** While this is a multi-system inflammatory vasculitis, its strongest genetic association is with **HLA-B51**, not HLA-B27. **High-Yield Clinical Pearls for NEET-PG:** * **Mnemonic for HLA-B27 (PEAR):** **P**soriatic arthritis, **E**nteropathic arthritis (IBD), **A**nkylosing spondylitis (strongest association >90%), and **R**eactive arthritis [1]. * **Reactive Arthritis** is most commonly triggered by *Chlamydia trachomatis* (urogenital) or *Shigella, Salmonella, Campylobacter* (enteric) [1][2]. * **Extra-articular features** of HLA-B27 conditions often include **Acute Anterior Uveitis** and **Aortitis** [1]. * **Keratoderma blennorrhagica** (skin lesions on palms/soles) is a pathognomonic finding in Reiter’s Syndrome [2].

Q: An elderly female presents with fever, headache, and diplopia. Biopsy of the artery revealed panarteritis. What is the most probable diagnosis?

Temporal arteritis. ### Explanation **Correct Answer: D. Temporal arteritis (Giant Cell Arteritis)** **Why it is correct:** Temporal arteritis (GCA) is a large-vessel vasculitis that characteristically affects elderly individuals (usually >50 years) [1]. The classic triad includes **fever, headache, and visual disturbances** (like diplopia or amaurosis fugax). The pathology reveals **panarteritis**—inflammation involving all layers of the arterial wall—often with giant cell formation and internal elastic lamina fragmentation [2]. Diplopia in GCA is a medical emergency, as it signifies ischemia to the extraocular muscles or cranial nerves, risking permanent blindness. **Why the other options are incorrect:** * **A. Nonspecific arteritis:** This is a vague term and not a recognized clinical entity for this specific presentation. * **B. Polyarteritis nodosa (PAN):** This is a medium-vessel vasculitis that typically spares the lungs and rarely presents with isolated headache/diplopia. It is strongly associated with Hepatitis B and presents with "rosary sign" (aneurysms) on angiography [1]. * **C. Wegener's granulomatosis (GPA):** This small-vessel vasculitis involves the triad of upper respiratory tract (sinusitis), lower respiratory tract (hemoptysis), and kidneys (GN). It is c-ANCA positive. **High-Yield Clinical Pearls for NEET-PG:** * **Gold Standard Diagnosis:** Temporal artery biopsy (look for "skip lesions") [2]. * **Lab Marker:** Markedly elevated **ESR** (often >100 mm/hr) and CRP [2]. * **Associated Condition:** Strongly linked with **Polymyalgia Rheumatica (PMR)**, characterized by proximal muscle stiffness. * **Treatment:** Immediate high-dose **Corticosteroids** (do not wait for biopsy results if vision is threatened) to prevent permanent vision loss [2]. * **Most common vessel involved:** Superficial temporal artery.

Q: What is the drug of choice for resistant macrophage activation syndrome?

Cyclosporine. **Explanation:** **Macrophage Activation Syndrome (MAS)** is a life-threatening complication of systemic inflammatory disorders (most commonly Systemic Juvenile Idiopathic Arthritis). It is considered a form of secondary Hemophagocytic Lymphohistiocytosis (HLH), characterized by a "cytokine storm," cytopenias, and multi-organ failure. **Why Cyclosporine is the Correct Answer:** While high-dose corticosteroids (Methylprednisolone) are the first-line treatment for MAS, **Cyclosporine (a calcineurin inhibitor)** is the **drug of choice for resistant or refractory cases**. It works by inhibiting T-cell activation and the subsequent production of pro-inflammatory cytokines (like IL-2 and IFN-gamma) that drive the inappropriate activation of macrophages. Its rapid onset of action and ability to dampen the "cytokine storm" make it the gold standard for steroid-resistant MAS. **Analysis of Incorrect Options:** * **A. Tacrolimus:** Although also a calcineurin inhibitor, it is less extensively studied than Cyclosporine for MAS and is not the standard recommendation for resistant cases. * **B. Methylprednisolone:** This is the **initial/first-line** treatment. The question specifically asks for the drug of choice in **resistant** cases. * **D. Tocilizumab:** While this IL-6 inhibitor is used in systemic JIA, its role in MAS is controversial. It may mask clinical features (like fever and CRP rise) and is generally not the preferred agent for acute, resistant MAS. **High-Yield Clinical Pearls for NEET-PG:** * **HLH/MAS Diagnostic Hallmark:** Extreme hyperferritinemia (often >10,000 ng/mL). * **HLH-2004 Protocol:** Often includes Etoposide, Dexamethasone, and **Cyclosporine**. * **Emerging Therapy:** **Anakinra** (IL-1 receptor antagonist) is increasingly used for refractory MAS due to its excellent safety profile. * **Key Lab Finding:** Paradoxically low or normal ESR (due to fibrinogen consumption) despite high clinical inflammation.

Q: Involvement of which of the following muscles virtually excludes the diagnosis of polymyositis?

Extraocular muscles. The hallmark of **Polymyositis (PM)** and **Dermatomyositis (DM)** is symmetric, subacute, progressive weakness of the **proximal limb muscles** (shoulders and hips) [1]. A crucial diagnostic feature of these inflammatory myopathies is the **sparing of the extraocular and facial muscles.** **Why Extraocular Muscles are the Correct Answer:** Polymyositis specifically targets skeletal muscles of the trunk and limbs but characteristically spares the extraocular muscles. If a patient presents with proximal muscle weakness accompanied by ptosis or diplopia (extraocular involvement), clinicians must look for alternative diagnoses such as **Myasthenia Gravis** or **Oculopharyngeal Muscular Dystrophy** [2]. **Analysis of Incorrect Options:** * **Neck Muscles:** Weakness of the neck flexors is very common in PM, often leading to "dropped head syndrome" or difficulty lifting the head from a pillow. * **Esophageal Muscles:** PM frequently involves the striated muscles of the upper third of the esophagus and the pharynx [1]. This leads to **dysphagia** and an increased risk of aspiration pneumonia. * **Abdominal Muscles:** As part of the core/trunk musculature, the abdominal muscles can be involved, contributing to difficulty rising from a supine position [1]. **NEET-PG High-Yield Pearls:** * **Sparing Rule:** PM/DM spares extraocular muscles (unlike Myasthenia Gravis) and distal muscles (unlike Inclusion Body Myositis). * **Enzymes:** Elevated **Creatine Kinase (CK)** is the most sensitive initial lab marker. * **Antibodies:** **Anti-Jo-1** (Histidyl-tRNA synthetase) is the most common myositis-specific antibody and is associated with "Mechanic’s hands," interstitial lung disease, and Raynaud's phenomenon (Antisynthetase Syndrome) [1]. * **Malignancy:** Dermatomyositis (more than PM) has a strong association with underlying occult malignancies (e.g., ovarian, lung, breast).

Q: Arthritis mutilans is seen in which condition?

Psoriatic arthropathy. **Explanation:** **Arthritis Mutilans** is the most severe and destructive form of inflammatory arthritis, characterized by extensive bone resorption (osteolysis) leading to the collapse of joint surfaces. **Why Psoriatic Arthropathy is Correct:** While several conditions can rarely cause joint destruction, **Psoriatic Arthritis (PsA)** is the classic and most common association cited in medical literature and exams. It occurs in about 5% of PsA patients. The hallmark is "telescoping fingers" (main-en-lorgnette), where the digits shorten because the bones have dissolved, and the overlying skin becomes redundant and wrinkled. Radiologically, this presents as the **"Pencil-in-cup" deformity**, where the proximal bone is whittled down (pencil) and the distal bone surface is excavated (cup). **Analysis of Incorrect Options:** * **Rheumatoid Arthritis (B):** While RA causes significant joint erosion and deformities (like ulnar deviation), it typically does not lead to the gross osteolysis and "telescoping" seen in true arthritis mutilans. * **Spondyloarthropathy (C):** This is a broad category (including Ankylosing Spondylitis). While PsA is a type of Seronegative Spondyloarthropathy, the term "Arthritis Mutilans" specifically refers to the peripheral joint destruction most unique to the Psoriatic subtype. * **Reactive Arthritis (D):** Usually presents as an asymmetric oligoarthritis of the lower limbs; it rarely progresses to the level of bone resorption seen in mutilans. **High-Yield Clinical Pearls for NEET-PG:** * **Radiological Sign:** "Pencil-in-cup" appearance is pathognomonic for Psoriatic Arthritis. * **Clinical Sign:** "Main-en-lorgnette" (Opera-glass hand). * **Other Associations:** Arthritis Mutilans can also be seen in severe cases of Leprosy (due to neurotrophic changes) and multicentric reticulohistiocytosis. * **Dermatology Link:** Look for nail changes (pitting, onycholysis) in patients with Psoriatic Arthritis.

Q: Which of the following is NOT a feature of Lofgren syndrome?

Uveitis. **Explanation:** Lofgren syndrome is a specific, acute clinical presentation of **Sarcoidosis** characterized by a classic triad. It typically has a benign course with a high rate of spontaneous remission [1]. **1. Why Uveitis is the correct answer:** While uveitis is a common manifestation of systemic sarcoidosis (occurring in about 25-50% of cases), it is **not** part of the defined diagnostic triad for Lofgren syndrome [1]. Lofgren syndrome is specifically defined by the presence of **Erythema Nodosum (EN), Bilateral Hilar Lymphadenopathy (BHL), and Joint involvement.** **2. Analysis of incorrect options:** * **B. Erythema nodosum:** This is a hallmark cutaneous finding of Lofgren syndrome, more commonly seen in women [1]. It presents as painful, erythematous nodules on the shins. * **C. Polyarthritis:** Migratory polyarthritis, particularly involving the **ankle joints**, is a core component [1]. In men, periarthritis of the ankles may sometimes occur in the absence of EN. * **D. Bilateral hilar adenopathy:** This is the classic radiographic finding required for diagnosis [1]. It is usually asymptomatic and found on a routine chest X-ray. **High-Yield Clinical Pearls for NEET-PG:** * **Prognosis:** Lofgren syndrome carries an excellent prognosis (>90% resolution within 2 years) compared to other forms of sarcoidosis. * **Heerfordt Syndrome (Uveoparotid Fever):** Do not confuse Lofgren with Heerfordt syndrome, which *does* include **Uveitis**, along with Parotid enlargement, Fever, and Facial nerve palsy. * **HLA Association:** Strongly associated with **HLA-DRB1*03**. * **Treatment:** Primarily symptomatic with NSAIDs; systemic steroids are rarely needed unless severe.

Question 1

Which of the following conditions is associated with HLA-B27?

Accepted Answer

Reiter's Syndrome

Answer

Osteoarthritis

Answer

Sjogren syndrome

Answer

Behcet disease

Question 2

An elderly female presents with fever, headache, and diplopia. Biopsy of the artery revealed panarteritis. What is the most probable diagnosis?

Accepted Answer

Temporal arteritis

Answer

Nonspecific arteritis

Answer

Polyarteritis nodosa (PAN)

Answer

Wegener's granulomatosis

Question 3

What is the drug of choice for resistant macrophage activation syndrome?

Accepted Answer

Cyclosporine

Answer

Tacrolimus

Answer

Methylprednisolone

Answer

Tocilizumab

Question 4

Involvement of which of the following muscles virtually excludes the diagnosis of polymyositis?

Accepted Answer

Extraocular muscles

Answer

Neck muscles

Answer

Esophageal muscles

Answer

Abdominal muscle

Question 5

Following a severe sore throat, a 15-year-old boy feels unwell. He has had pain and swelling in his elbows and knees, and cardiac examination reveals new mitral regurgitation and tachycardia. Which of the following might also be seen on further examination?

Accepted Answer

Subcutaneous nodules

Answer

Chronic arthritis

Answer

Involvement of spinal joints

Answer

Erythema nodosum

Question 6

A patient with dermatomyositis shows improvement of symptoms on steroids for the last 6 months. Labs today show a Creatine kinase of 1300 Units/L. What is the next step in the management?

Accepted Answer

Continue the same dose of steroids

Answer

Raise the dose of steroids

Answer

Start mycophenolate

Answer

Perform muscle biopsy

Question 7

Arthritis mutilans is seen in which condition?

Accepted Answer

Psoriatic arthropathy

Answer

Rheumatoid arthritis

Answer

Spondyloarthropathy

Answer

Reactive arthritis

Question 8

Which of the following is NOT a feature of Lofgren syndrome?

Accepted Answer

Uveitis

Answer

Erythema nodosum

Answer

Polyarthritis

Answer

Bilateral hilar adenopathy

Question 9

An 80-year-old female presented with soreness in both shoulders and upper back, constant fatigue, and persistent headaches for the past few days. She was managed conservatively. Three days later, she presented with sudden loss of vision in the left eye. Ophthalmoscopic examination was performed. What is the next best appropriate step in the management of this condition?

Accepted Answer

Initiate high-dose glucocorticoid therapy and refer for temporal artery biopsy

Answer

Brain MRI to evaluate for demyelinating disease or tumor

Answer

Ocular massage for 2 minutes followed by repeat visual acuity testing

Answer

Topical beta-blocker administration

Question 10

A 30-year-old woman presents with hemoptysis, epistaxis, and decreased urine output. Nasal mucosal ulcers were seen on inspection. Chest X-ray shows three cavitary lesions. She is positive for ANCA. What is the most likely diagnosis?

Accepted Answer

Wegener's granulomatosis

Answer

Goodpasture's syndrome

Answer

Berger's nephropathy

Answer

Alport's disease

Rheumatology and Immunology — MCQs

Rheumatology and Immunology — MCQs

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