Rheumatology and Immunology Practice Questions

Q: What is the best initial treatment for a patient with Behcet syndrome and ocular involvement?

Systemic glucocorticoids and azathioprine. Behcet syndrome is a multisystem inflammatory vasculitis characterized by recurrent oral and genital ulcers, skin lesions, and uveitis. The management strategy is dictated by the severity of organ involvement. **Why Systemic Glucocorticoids and Azathioprine are correct:** Ocular involvement (typically posterior uveitis or panuveitis) is considered a "major organ" manifestation that carries a high risk of permanent vision loss. Therefore, aggressive systemic therapy is required. **Systemic glucocorticoids** provide rapid control of acute inflammation, while **Azathioprine** is the preferred first-line steroid-sparing immunosuppressant to prevent relapses and long-term ocular damage. In severe or refractory cases, anti-TNF agents (Infliximab) or Cyclosporine may be used. **Why other options are incorrect:** * **Option A:** Topical steroids are sufficient for isolated minor oral ulcers or anterior uveitis, but they cannot penetrate the posterior segment of the eye or prevent systemic progression. * **Option B:** Thalidomide is effective for refractory mucocutaneous ulcers but is not the standard of care for ocular disease due to its side effect profile and lack of efficacy in preventing blindness. * **Option D:** Interferon-alfa is a second-line or adjunctive therapy for resistant cases; it is not the "best initial" treatment. **High-Yield Clinical Pearls for NEET-PG:** * **Pathergy Test:** A highly specific (but less sensitive) diagnostic test where a sterile needle prick results in a papule/pustule within 24–48 hours. * **HLA Association:** Strongly associated with **HLA-B51**. * **Classic Triad:** Recurrent oral ulcers, genital ulcers, and uveitis. * **Vascular involvement:** Behcet is unique as it involves both arteries and veins (e.g., Budd-Chiari syndrome, pulmonary artery aneurysms).

Q: What is the most common lymphoma associated with Sicca syndrome?

Mucosa-associated lymphoid tissue lymphoma (MALToma). Sicca syndrome (primary Sjögren’s syndrome) is characterized by chronic lymphocytic infiltration of the exocrine glands. Patients with Sjögren’s syndrome have a **44-fold increased risk** of developing non-Hodgkin lymphoma compared to the general population. **1. Why MALToma is correct:** The chronic antigenic stimulation of B-cells within the salivary glands leads to the formation of ectopic germinal centers. Over time, this persistent activation can result in a monoclonal B-cell proliferation, most commonly leading to **Extranodal Marginal Zone B-cell Lymphoma**, also known as **MALToma** [1]. These typically arise within the parotid glands. **2. Analysis of Incorrect Options:** * **Diffuse large B-cell lymphoma (DLBCL):** While Sjögren’s patients can develop DLBCL, it is usually a result of a high-grade transformation from a pre-existing MALToma. It is not the *most common* initial presentation. * **Burkitt lymphoma:** This is an aggressive B-cell lymphoma associated with EBV and c-myc translocation; it is not specifically linked to the chronic inflammatory environment of Sicca syndrome [1]. * **Lymphoplasmacytic lymphoma:** This is associated with Waldenström macroglobulinemia. While Sjögren’s involves plasma cell activity, this specific lymphoma is not the characteristic association. **High-Yield Clinical Pearls for NEET-PG:** * **Predictors of Lymphoma in Sjögren’s:** Persistent parotid gland enlargement, purpura, leukopenia, cryoglobulinemia, and low C4 complement levels. * **Most common site:** The parotid gland is the most frequent site for lymphoma development in these patients. * **Biomarker:** A decrease in previously high rheumatoid factor (RF) titers or the disappearance of hypergammaglobulinemia can paradoxically signal the onset of lymphoma.

Q: An 18-year-old boy presents with digital gangrene in the third and fourth fingers for the last 2 weeks. On examination, the blood pressure is 170/110 mm of Hg, and all peripheral pulses were palpable. Food and urine examinations were unremarkable. Antinuclear antibodies, antibody to double-stranded DNA, and antineutrophil cytoplasmic antibody were negative. What is the most likely diagnosis?

Polyarteritis nodosa. The clinical presentation of **Polyarteritis Nodosa (PAN)** is characterized by a medium-vessel vasculitis that typically spares the lungs and the glomeruli [1]. **Why Polyarteritis Nodosa (PAN) is correct:** 1. **Digital Gangrene:** PAN involves medium-sized arteries, leading to ischemia and infarction of distal extremities [2]. 2. **Hypertension:** This is a hallmark of PAN, resulting from renal artery involvement (renin-mediated) rather than glomerulonephritis. 3. **Palpable Pulses:** Unlike large-vessel vasculitis, peripheral pulses are usually preserved because the primary pathology is in smaller branch arteries. 4. **Negative Serology:** PAN is characteristically **ANCA-negative** (unlike Wegener’s) and **ANA/dsDNA-negative** (unlike SLE). The "unremarkable urine" confirms the absence of glomerulonephritis, a key diagnostic feature that distinguishes PAN from microscopic polyangiitis. **Why other options are incorrect:** * **Wegener’s Granulomatosis (GPA):** Typically presents with upper/lower respiratory tract involvement and is strongly associated with **c-ANCA (PR3)**. * **Takayasu’s Arteritis:** A large-vessel vasculitis that usually presents with **absent/diminished pulses** ("pulseless disease") and is more common in young females. * **Systemic Lupus Erythematosus (SLE):** While it can cause vasculitis, the negative ANA and dsDNA, along with the absence of proteinuria/hematuria, make it highly unlikely. **High-Yield Clinical Pearls for NEET-PG:** * **PAN Association:** Strongly associated with **Hepatitis B** (HBV) surface antigenemia (10–30% of cases). * **Angiography:** Shows characteristic **"string of pearls"** appearance due to multiple microaneurysms. * **Sparing:** PAN classically **spares the lungs** (no pulmonary involvement). * **Biopsy:** Gold standard for diagnosis, showing transmural necrotizing inflammation.

Q: Which of the following findings is NOT seen in Dermatomyositis?

Salmon rash. **Explanation:** The correct answer is **Salmon rash**, as this is a characteristic clinical finding of **Adult-Onset Still’s Disease (AOSD)**, not Dermatomyositis. The salmon-colored, maculopapular, non-pruritic rash typically appears during febrile spikes on the trunk and limbs. **Why the other options are incorrect (Findings in Dermatomyositis):** * **Gottron’s papules (Option A):** These are pathognomonic for Dermatomyositis [1]. They are erythematous to violaceous scaly papules found over the dorsal aspects of the MCP, PIP, and DIP joints [2]. * **Mechanic’s hands (Option B):** Characterized by hyperkeratotic, fissured, and scaling skin on the lateral and palmar aspects of the fingers. While seen in Dermatomyositis, it is highly associated with **Anti-synthetase syndrome** (Anti-Jo-1 antibodies) [1]. * **Periungual telangiectasia (Option C):** These are dilated capillary loops at the nail folds, often accompanied by cuticular hypertrophy [2]. They reflect the underlying systemic microvascular involvement of the disease. **High-Yield Clinical Pearls for NEET-PG:** 1. **Heliotrope Rash:** A violaceous eruption on the upper eyelids, often with periorbital edema; another pathognomonic sign [2]. 2. **Shawl Sign & V-sign:** Photosensitive erythematous rashes over the posterior neck/shoulders and the anterior chest, respectively [2]. 3. **Malignancy Risk:** Dermatomyositis has a strong association with internal malignancies (e.g., ovarian, lung, breast, GI); age-appropriate cancer screening is mandatory [2]. 4. **Antibody Profile:** **Anti-Mi-2** is highly specific for Dermatomyositis and carries a good prognosis.

Q: Interstitial lung disease is seen in which of the following conditions?

Diffuse systemic sclerosis. **Explanation:** Interstitial Lung Disease (ILD) is a common pulmonary manifestation of various Connective Tissue Diseases (CTDs), but its prevalence and clinical significance vary significantly across conditions [1]. **Why Diffuse Systemic Sclerosis (SSc) is the correct answer:** ILD is a hallmark feature of Systemic Sclerosis, particularly the **Diffuse Cutaneous subtype**. It is the leading cause of mortality in these patients. Approximately 70-90% of SSc patients show evidence of ILD on HRCT, with **NSIP (Non-Specific Interstitial Pneumonia)** being the most common histological pattern [1]. It typically presents with exertional dyspnea and "velcro" crackles on auscultation [1]. **Analysis of Incorrect Options:** * **Polymyositis (PM):** While ILD occurs in PM (especially in Anti-Jo-1 positive/Antisynthetase syndrome), it is less frequent than in SSc. * **Rheumatoid Arthritis (RA):** ILD is a well-known extra-articular manifestation of RA (typically the **UIP pattern**), but it occurs in a smaller subset of patients (approx. 10%) compared to the near-universal involvement in diffuse SSc [1]. * **Systemic Lupus Erythematosus (SLE):** Pulmonary involvement in SLE most commonly manifests as **Pleuritis** (pleural effusion). While ILD can occur, it is rare compared to SSc, RA, or Myositis [1]. **High-Yield Clinical Pearls for NEET-PG:** * **Most common cause of death in SSc:** ILD (previously it was Scleroderma Renal Crisis before ACE inhibitors). * **Specific Marker:** Anti-Scl-70 (Anti-topoisomerase I) is strongly associated with ILD in SSc. * **Drug of Choice:** Mycophenolate Mofetil (MMF) is now preferred over Cyclophosphamide for SSc-ILD. * **Pattern Recognition:** SSc is associated with **NSIP**, whereas RA is more commonly associated with **UIP** [1].

Q: Leukopenia in Systemic Lupus Erythematosus (SLE) is almost always associated with which specific cell type deficiency?

Lymphopenia. In Systemic Lupus Erythematosus (SLE), hematological abnormalities are a hallmark of the disease and are included in the ACR/SLICC classification criteria. **Lymphopenia** (defined as <1,500/mm³) is the most characteristic and frequent cause of leukopenia in SLE [1]. **1. Why Lymphopenia is the correct answer:** Lymphopenia in SLE is primarily mediated by **anti-lymphocyte antibodies** (Type II hypersensitivity). These autoantibodies target surface antigens on T and B cells, leading to their destruction or sequestration. It is a highly specific marker of disease activity; a falling lymphocyte count often correlates with an impending SLE flare [1]. **2. Why other options are incorrect:** * **Neutropenia:** While it can occur in SLE, it is much less common than lymphopenia. When present, it is often mild or related to medications (e.g., immunosuppressants) rather than the primary disease process itself. * **Eosinopenia:** This is not a characteristic feature of SLE. Eosinopenia is more commonly associated with acute stress, Cushing’s syndrome, or the use of glucocorticoids. * **Any of the above:** While multiple cell lines can be affected (pancytopenia), lymphopenia is the "almost always" associated finding that defines the leukopenic state in the context of SLE diagnostic criteria [1]. **Clinical Pearls for NEET-PG:** * **Hematological Criteria for SLE:** Leukopenia (<4,000/mm³), Lymphopenia (<1,500/mm³), Thrombocytopenia (<100,000/mm³), and Autoimmune Hemolytic Anemia [1]. * **Anemia of Chronic Disease** is the most common cause of anemia in SLE, but **Autoimmune Hemolytic Anemia (Coombs positive)** is the one included in the diagnostic criteria. * **High-Yield Fact:** If a patient with SLE presents with sudden, severe neutropenia, always rule out **Felty’s Syndrome** (though more common in RA) or drug-induced marrow suppression.

Q: Grottron's sign is a feature of which of the following conditions?

Dermatomyositis. **Dermatomyositis** is an idiopathic inflammatory myopathy characterized by proximal muscle weakness and distinctive cutaneous manifestations [1]. **Gottron’s sign** is a pathognomonic feature of this condition [1],[2]. It presents as symmetric, erythematous to violaceous macules or patches overlying the dorsal aspect of the interphalangeal and metacarpophalangeal joints [2]. When these lesions become raised, they are referred to as **Gottron’s papules** [1],[2]. **Why other options are incorrect:** * **Systemic Sclerosis (Scleroderma):** Characterized by skin thickening (sclerodactyly), Raynaud’s phenomenon, and digital pitting scars, but not Gottron’s sign. * **Systemic Lupus Erythematosus (SLE):** Features a malar (butterfly) rash. Notably, SLE rashes typically **spare the knuckles** (interarticular areas), whereas Dermatomyositis specifically involves the skin over the joints. * **Sjögren’s Syndrome:** Primarily presents with sicca symptoms (dry eyes and dry mouth) due to lymphocytic infiltration of exocrine glands. **High-Yield Clinical Pearls for NEET-PG:** * **Heliotrope Rash:** A violaceous eruption on the upper eyelids, often with periorbital edema; also pathognomonic for Dermatomyositis [2]. * **Shawl Sign & V-sign:** Photosensitive erythematous rashes on the back/shoulders and anterior chest, respectively [2]. * **Mechanic’s Hands:** Hyperkeratotic, fissured skin on the lateral fingers (associated with Anti-Jo-1 antibodies) [1]. * **Malignancy Link:** Dermatomyositis in adults is frequently associated with an increased risk of internal malignancies (e.g., ovarian, lung, breast) [2]. * **Diagnosis:** Elevated Creatine Kinase (CK), electromyography (EMG) changes, and definitive muscle biopsy showing **perifascicular atrophy**.

Q: What is the most common presentation of Systemic Lupus Erythematosus (SLE)?

Arthralgia. **Explanation:** Systemic Lupus Erythematosus (SLE) is a multisystem autoimmune disease characterized by the production of autoantibodies [1]. While SLE is famous for its dermatological manifestations, **musculoskeletal involvement** is the most frequent clinical feature, occurring in over 90% of patients during the course of the disease [1]. * **Why Arthralgia is Correct:** Arthralgia (joint pain) or non-erosive arthritis is the **most common presenting symptom** of SLE. It typically affects the small joints of the hands, wrists, and knees in a symmetrical pattern. Unlike Rheumatoid Arthritis, the joint involvement in SLE is generally non-destructive. * **Why Erosive Polyarthritis is Incorrect:** SLE is classically characterized by **non-erosive** arthritis. While "Jaccoud’s arthropathy" (reducible deformities due to lax ligaments) can occur, true bone erosions are rare and suggest "Rhupus" (an overlap of RA and SLE). * **Why Malar Rash is Incorrect:** Although the "butterfly rash" is the most specific and iconic sign of SLE, it occurs in approximately 50–60% of patients, making it less common than joint pain [1]. * **Why Autoimmune Hemolytic Anemia (AIHA) is Incorrect:** Hematologic abnormalities are common (especially Anemia of Chronic Disease and Leukopenia), but overt AIHA occurs in only about 10% of patients. **NEET-PG High-Yield Pearls:** * **Most common symptom:** Fatigue. * **Most common clinical sign:** Arthralgia/Arthritis. * **Most common renal lesion:** Diffuse Proliferative Glomerulonephritis (Class IV). * **Most common cardiac manifestation:** Pericarditis. * **Best Screening Test:** ANA (High sensitivity). * **Most Specific Test:** Anti-dsDNA or Anti-Smith antibodies.

Question 1

What is the best initial treatment for a patient with Behcet syndrome and ocular involvement?

Accepted Answer

Systemic glucocorticoids and azathioprine

Answer

Topical glucocorticoids to the oral ulcers and conjunctiva

Answer

Thalidomide

Answer

Intralesional interferon alfa

Question 2

What is the most common lymphoma associated with Sicca syndrome?

Accepted Answer

Mucosa-associated lymphoid tissue lymphoma (MALToma)

Answer

Diffuse large B-cell lymphoma (DLBCL)

Answer

Burkitt lymphoma

Answer

Lymphoplasmacytic lymphoma

Question 3

An 18-year-old boy presents with digital gangrene in the third and fourth fingers for the last 2 weeks. On examination, the blood pressure is 170/110 mm of Hg, and all peripheral pulses were palpable. Food and urine examinations were unremarkable. Antinuclear antibodies, antibody to double-stranded DNA, and antineutrophil cytoplasmic antibody were negative. What is the most likely diagnosis?

Accepted Answer

Polyarteritis nodosa

Answer

Wegener's granulomatosis

Answer

Takayasu's arteritis

Answer

Systemic lupus erythematosus (SLE)

Question 4

Which of the following findings is NOT seen in Dermatomyositis?

Accepted Answer

Salmon rash

Answer

Gottron's papules

Answer

Mechanic's hands

Answer

Periungual telangiectasia

Question 5

Interstitial lung disease is seen in which of the following conditions?

Accepted Answer

Diffuse systemic sclerosis

Answer

Polymyositis

Answer

Rheumatoid arthritis

Answer

Systemic lupus erythematosus

Question 6

Raynaud's phenomenon is seen in which of the following conditions?

Accepted Answer

Scleroderma

Answer

Reiter's disease

Answer

Rheumatoid arthritis

Answer

Behcet's syndrome

Question 7

Leukopenia in Systemic Lupus Erythematosus (SLE) is almost always associated with which specific cell type deficiency?

Accepted Answer

Lymphopenia

Answer

Neutropenia

Answer

Eosinopenia

Answer

Any of the above

Question 8

Grottron's sign is a feature of which of the following conditions?

Accepted Answer

Dermatomyositis

Answer

Systemic sclerosis

Answer

Systemic lupus erythematosus

Answer

Sjögren's syndrome

Question 9

What is the most common presentation of Systemic Lupus Erythematosus (SLE)?

Accepted Answer

Arthralgia

Answer

Erosive polyarthritis

Answer

Malar rash

Answer

Autoimmune hemolytic anemia

Question 10

Which of the following immunoglobulins are used in the treatment of Myasthenia gravis, Idiopathic Thrombocytopenic Purpura, and Guillain-Barré syndrome?

Accepted Answer

Myasthenia gravis, Idiopathic Thrombocytopenic Purpura, Guillain-Barré syndrome

Answer

Myasthenia gravis, Idiopathic Thrombocytopenic Purpura, Polyarteritis Nodosa

Answer

Idiopathic Thrombocytopenic Purpura, Polyarteritis Nodosa, Wegener's Granulomatosis

Answer

Polyarteritis Nodosa, Wegener's Granulomatosis, Guillain-Barré syndrome

Rheumatology and Immunology — MCQs

Rheumatology and Immunology — MCQs

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